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Motel Freehold Going Concern for Sale Central Otago
Motel freehold going concern for sale now! Easy to manage 5 motel units and lovely 3 bedroom home. Exciting opportunity to build additional motel units and manager's residence in the future.**UPDATE** Latest Rating Valuation CV of $720,000 for house and motel. Price reduced as our Vendor's are motivated to sell as they are looking to retire and hand over to the new operators!
+GST if any - Price Reduced!
Business for Sale Description
Motel Freehold Going Concern for Sale Central Otago
Big motel buying opportunity here for the price!
Motel sale in Central Otago New Zealand - Great Tourism Accommodation Freehold Going Concern Land, Buildings, Chattels, Goodwill and Business where all the hard work done, great owners accommodation, great room configuration and layout to acheive great occupancy yields.
Resource Consent: Resource consent is in place with plans to build an additional 4 motel units and Managers Residence which will lapse on 17 April 2018.
With the strenght of the tourism industry in New Zealand this could be a very appealing option in the furture.
Notice of Rating Valuation as at 1 September 2016 are below:
- House: $235,000
- Motel: $485,000
Business Resources
The motel units are fully self-contained, they have been refurbished to a high standard with kitchen facilities and all units have their own covered carports. There is a recreational and barbecue area, outside seating with umbrellas for summer dining, secure lock up available for cyclists and laundry.
Excellent location and only minutes walk to cafes, restaurants, shops etc and a meeting place of two of the most popular Rail Trails.
Central Otago has a good climate with outstanding scenery and offers a fantastic lifestyle.
I invite all interested parties to contact me to discuss this sale now.


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Find your new home in Adna, WAYou are viewing homes 1 to 1 of 1 home for sale
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Learn more about homes in Adna, WA
Adna Home inventory breakdown by property type
Of the 1 homes for sale in Adna, the breakdown of property types
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100% Land/lot.
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You are Viewing 1 to 1 of 1 homes for sale in Adna. These homes are comprised of Land/lot. The average list price of these property results is 50,000. Prices are displayed as US Dollar. For more information on these homes for sale in Adna please contact our member brokerages to schedule a showing or view an open house.
The 1 homes for sale in Adna, WA are represented by 1 member brokerages serving the Adna area. Member brokerages representing the 1 homes include Windermere Relocation - Seattle.

subset: commoncrawl


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MTV2 is having a Muse night tonight, and will be broadcasting the Shepherd's Bush Gig from 11pm.
Tom Kirk
Hey. Its Muse night on MTV2 tonight. Videos and interviews and the like from 9pm. The main feature of the evening is the Shepherds Bush gig which is going out at 11pm. They will be beaming out nine tracks in total, it was a great gig and looks hot in the edit. Good on all those that entered and made it along. Sorry to those that didnt win. Next time I nearly promise you will. Cheers. Enjoy.
Only 9 tracks, and probably the 9 we already have.


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Miami at Purdue
Northwestern at Georgia Tech
Illinois at Maryland
Michigan at Virginia
Clemson at Iowa
Duke at Ohio State
Wednesday, November 30
Indiana at NC State
Penn State at Boston College
Florida State at Michigan State
Virginia Tech at Minnesota
Wake Forest at Nebraska
Wisconsin at North Carolina
(Times and networks are to be determined)
UNC Press Release:
CHAPEL HILL - The University of North Carolina will play Wisconsin in the 2011 Big Ten/ACC Challenge on Wednesday November 30, the Atlantic Coast Conference announced on Tuesday afternoon. The 13th annual event on Nov. 29-30 will be highlighted by the Badgers' first game in Chapel Hill and Duke at Ohio State -- four traditional power programs expected to be highly ranked entering the 2011-12 season - and an expanded format with new Big Ten member Nebraska. ESPN, ESPN2, ESPNU and ESPN3.com will combine to televise all 12 games of the two-day event, one more than in the previous 12 Challenges.
The 2011 Challenge involves six teams ranked an early preseason top 25 by ESPN.com senior college basketball writer Andy Katz, including three in the top five: No. 1 Carolina, No. 3 Ohio State, No. 5 Duke, No. 11 Wisconsin, No. 21 Florida State and No. 23 Michigan.
This year's event - top college basketball programs playing for conference supremacy and the Commissioner's Cup -- will include 11 teams that played in the 2011 NCAA Tournament: Clemson, Duke, Florida State and North Carolina from the ACC, and Illinois, Michigan, Michigan State, Ohio State, Penn State, Purdue and Wisconsin from the Big Ten.
Since 2000, the ACC (10) and the Big Ten (nine) have combined for 19 Final Four appearances and six national titles: UNC (2005 and 2009), Maryland (2002), Duke (2001 and 2010) and Michigan State (2000). The ACC leads in NCAA Tournament victories and Final Four appearances while the Big Ten is first in Tournament appearances.
North Carolina in the Big Ten/ACC Challenge:
- 12 appearances, 6-6 record
- This will be the first meeting in the ACC-Big Ten Challenge between North Carolina and Wisconsin Carolina and Wisconsin have only played one previous game - the Tar Heels beat the Badgers, 88-82, on March 27, 2005, in Syracuse, N.C., in the NCAA East Regional final. UNC won Roy Williams' first national championship the next weekend in St. Louis, Mo.
- UNC is 2-3 at home, 2-3 on the road and 2-0 at neutral sites (Greensboro and Detroit) in the ACC-Big Ten Challenge
- UNC is 2-0 vs. Ohio State, 2-2 vs. Michigan State, 1-1 vs. Indiana and 1-3 vs. Illinois) in the ACC-Big Ten Challenge
- Carolina head coach Roy Williams is 6-2 in the ACC-Big Ten Challenge


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I love Vampire Hunter D Bloodlust but I wish there'd been more on the relationship between Meier Link and Charlotte so I am writing a sort of sequel just for them. It's about their experiences going to and at the City of the Night.
Note I do not own Vampire Hunter D or the characters Meier and Charlotte.
Thoughts are written in Italics.
Save Her
Meier Link ran onto the ship and into the control room. Gently he set down his beloved Charlotte in the passenger seat and took his own position. Quickly starting up the engines he forced the ancient ship to its limits in a desperate attempt to get free from the collapsing structure that was Carmela's palace. The vessel slowly began to move upward and as their altitude increased so did their speed. Blessed be the ship still operates, thought Meier. Meier wasn't necessarily a pilot so he set the ship on autopilot for the City of the Night.
That taken care of Meier reached for Charlotte and carried her to the ships infirmary that was built for the instance that a human partner be injured. She was dead. Had Meier's heart beat it would have stopped the second hers did but he couldn't give up. As he laid her cold body on an operating table he couldn't breathe. To see her like this… it killed him inside. Oh Charlotte, I am so sorry! Meier reached into the medical cabinet and pulled out an emergency bag of blood that could be used in emergencies, such as if a vampire's control slipped. He set up an intravenous solution and inserted the connected needed into Charlottes arm. Meier knew that a human could be revived within a certain time period after death. He hoped that he could revive Charlotte and with the blood supply she could recover.
He just had to restart her heart. Meier found the defibrillator in another cupboard and prepared to shock Charlotte. By exposing her body to volts of electricity the main organs could be restarted. "I apologize my love but it's necessary if we are going to be together. I cannot lose you!" As he said this Meier rubbed the two paddles together and thrust them upon her chest. Charlotte's body arched and thumped back onto the table. Meier removed the paddles and checked her vitals. Nothing.
"Come on Charlotte. For me, come back for me, please!" Again, he thrust the paddles to her flesh and again her body jumped as a reaction. HE checked again, still nothing. No! It hasn't been too long. She can still be saved she's got to! "Charlotte!" He screamed. Tears began to leak from his eyes as he tried again to revive his only love, his heart, and his life. Upon contact Charlotte's body reacted the same as before. Meier desperately checked for signs of life. There! She had a pulse! It was faint but he could feel it beneath his stone cold fingers! "Oh Charlotte thank heavens" Hem pulled her into a hug. She was still unconscious because of the blood lose but after some time the transfusion would bring her out of it.
After rechecking to make sure the fluids were hooked up correctly Meier pulled forward a chair and took a seat next to Charlotte. For the next few hours Meier kept careful vigil and waited for his human to awake.
A Number of Hours Later:
Meier's eyes had begun to slide closed as fatigue set in. His battle with D and emotional trauma of almost losing Charlotte took their toll on him. He wanted to sleep but he wanted to be there when Charlotte awoke. And so he barely managed to stay focused as he watched charlottes face. At first he had worried that she still might not awake from such a deep slumber but recently he face twitched every now and then. Suddenly a moan escaped her lips. Meier's head snapped to attention.
"mmmmmm" she moaned again. "Charlotte, Charlotte can you her me dear?" Meier asked.
"Meier" She whispered, eyes still closed. "Oh Charlotte I'm here" he clasped her hand and brought it to her face and kissed her knuckle then rubbed the back of her hand against his cheek. "I'm here". It was obvious she was waking up and so Meier removed the needle from her arm and set it aside. Then he refocused on her face. He cupped her face in his hand.
At this her eyes snapped open and focused on Meier's face. "Meier?" She said hesitantly like she almost didn't recognize him. "Yes my love it's me." "Oh Meier!" she said enthusiastically and sat up suddenly and hugged him around his neck. "Meier, my Meier, oh Meier" she kept repeating. He gladly pulled her into his arms and hugged her back. "I'm here Charlotte, I'm not going anywhere! I promise!" He said. Her grip around him tightened and then she released him. She pulled back to look him in the eyes. Tears had begun to stain her still pale cheeks. "You saved me" she said.
Meier hung his head in shame. "No my dear, I didn't. I wasn't strong enough. I wasn't quick enough." He raised his head to look at her again. "You died." All she did was nod. "I know" He looked at her in confusion. "After you held me I was consumed by darkness. Everything simply ended. I was floating in a type of nothingness. I don't think I had fully succumbed to death yet but I had at least fallen into a limbo. And then everything changed. The world I was in was flooded with light. And then I heard you voice. I ran towards it. I called to you and called and called. And then it was almost like I ran right back into my body. Suddenly I could feel my limbs again, my lungs as they filled with air. I was too weak to move but I could feel again. And then you voice found me again and I managed the strength to open my eyes and I met the most beautiful sight, you." She smiled as she finished and could not help but return the smile. "You did save me Meier. You brought me back." He quickly pulled her into a hug again. He truly could not live without this wonderful creature.
"Come" he said. "You will need more blood transfusion to regain your strength but for now you need rest. Let us retire". She nodded "Alright". Meier then picked her up bridal stile and carried her to the ships master bedroom. She would be sleeping with him in the main coffin. If she preferred otherwise she could ask for it tomorrow but for now she was sleeping with Meier no matter what. She wasn't leaving his side. He opened the lid with one hand and laid Charlotte in the coffin being as gentle as possible. Then he got in himself. After lying down he pulled the lid closed. It surprised him when after everything Charlotte still moved closer and cuddled against him. This made him very happy and he smiled as he wrapped an arm around her slender body. She sighed happily and he smiled wider.
As Meier closed his eyes to welcome sleep Charlotte's steady heart beat reminded him that he hadn't eaten since many days before. He was hungry. But unlike before when Charlotte was this close he did not feel the temptation to bite her. Her body was pressed so closed to hers he could feel the blood pump through her veins with every heart beat and yet his fangs did not elongate. Seeing her die once had truly shocked his system. He doubted he would be able to resist her forever but despite the hunger gnawing at him now his only need was to hold Charlotte and be with her.
Despite the attempts of humans, damphirs, and vampires. Finally, they were together, at peace, high within the stars.
** Well what do you think? Should I leave it as is or continue? Please Review Review Review!**


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that is so unbelievably horrible I do not even know where to begin
This is a discussion on Papers please, oops!!! within the Off Topic & Humor Discussion forums, part of the The Back Porch category; I hope this isn't too political. I don't mean it that way. And certainly not in a partisan way. Here's the link to the story ...
I hope this isn't too political. I don't mean it that way. And certainly not in a partisan way.
Here's the link to the story of a guy who was born here, Houston, had a birth certificate on him, had a TX DL on him, got turned over to CBP by the local cops.
CBP questioned him for many hours and eventually wore him down into confessing to be here illegally and to sign away his rights and agree to be deported.
According to the story, the same fate would have happened to his brother except that he happened to have a copy of a passport application on him and a selective service registration card (which really shows nothing about status). [Nowadays, how many 18 + men even do what they are supposed to do, and how many actually keep the documentation? Now there's about 25 million we could stop wasting, maintaining that anachronistic bureaucracy.]
They kept saying, These are not your documents. Youre lying to us. Youre going to go prison for 20 years, Torres said. They basically wore him down. Hes a 19-year-old kid.
As I've posted elsewhere, incidents like this happen about 300 times a year.
They are a good reason for us to give some real additional thought to creation of a papers please society.
In the story it is mentioned that "his brother located immigration lawyer Torres, who took the case pro bono."
Uh, isn't it sad that US Citizens can end up needing to prove themselves to disbelieving "officials."
I don't know an answer, a way forward, but we need the first step. We need to get back to the day when people didn't need to walk around with papers. Instead, we are moving in the opposite and wrong direction.
that is so unbelievably horrible I do not even know where to begin
"The value you put on the lost will be determined by the sacrifice you are willing to make to seek them until they are found."
As another DC member recently posted, "the plural of anecdote is not evidence."
In the spirit of balance, we also need to move in the direction of those who are required to carry papers (e.g., green card and visa holders) actually having them on their person as required. Let's not deny US citizens any of their rights, but let's also not give the wrong people a pass. "Trust, but verify."
AZCDL Life Member
NRA Patron Member
NROI Chief Range Officer
The time may soon come where I will be ticketed for not carrying my DL. But an illegal will be let go, no ticket, no detention, no questions. It's already happening in Charlotte, NC. Illegals have been allowed to leave the scene of a minor traffic accident, no DL, no insurance, and no ticket. Do you think that would happen for you or me? There is quickly becoming 2 Americas. One where citizens are held accountable, and one where illegals are not. And that needs to stop as well.
Do I think it's OK for a citizen to go through a lot of hassles to prove themselves legal? No. But I don't think it's too much to expect someone to have a DL or state ID that's valid or suffer legal consequences. I have to, why should they not? I also think the LEO's that apparently did not check his documents should be treated with little mercy. If it was possible to verify them, and they did not, they should be fired.
I prefer to live dangerously free than safely caged!
"Our houses are protected by the good Lord and a gun. And you might meet 'em both if you show up here not welcome son." Josh Thompson "Way Out Here"
That's the simple truth of any "official" claim leveled against anyone, pretty much. Officialdom has become so sacrosanct in its own mind that the Constitutionally supported innocence until proven guilty has been effectively morphed into guilty unless claims can be turned aside. We allow it to occur, by failing to have severe and costly "teeth" in our legal response to such "officials" who dare screw innocents with this heinous type of power play. Ten to one such criminals merely get fired, at best (if that; if anything other than being forced to say "oops" in reconciliation). In such clear cases of presumed guilt and harassment, I'd like to see fairly harsh fines and sentences for gross abuse of power against such people who dare level such things against innocents. Until then, it will continue.Uh, isn't it sad that US Citizens can end up needing to prove themselves to disbelieving "officials."
Your best weapon is your brain. Don't leave home without it.
Thoughts: Justifiable self defense (A.O.J.).
Explain: How does disarming victims reduce the number of victims?
Reason over Force: Why the Gun is Civilization (Marko Kloos).
It is astonishing. It's also a good reminder that just because certain "laws" are on the books doesn't really mean anything. What does matter is which laws are enforced and which are ignored.It's utterly amazing to me that illegals are not immediately deported each and every time they're found (after serving whatever sentences they might be convicted of, first, of course.)
If you remember the Hiibel vs Nevada case, then you're old enough to remember when a cop couldn't necessarily get up in your face and start demanding to "see some ID." There was a time in our country where, if you were doing nothing wrong, you could tell said cop to "buzz off." (Of course, there was an even earlier time where said cop would have thereupon proceeded to take you apart with a nightstick, but I digress.)
Over time, as we've gotten used to showing ID at airports, at the grocery store when using credit cards, it's easy to forget that this is a relatively new phenomenon. There's something kind of insulting and irritating at having a convenience store clerk ask you for ID: Who the hell are you and what right do you have to ask me to identify? Screw you!
A certain kind of American will read the above, nod his head and understand exactly what I'm talking about.
Another type will squint and feel uncomfortable: "You have done nothing wrong, so why not show ID?"
And yet another will think, "Dude, it's just ID - what's the problem?"
So. While we would like to see illegal aliens treated like the border-jumping scofflaws that they are, we should also be very careful about authorizing the police to conduct paperwork shakedowns at their own discretion. Maybe Officer Donut has a highly tuned sense of who is illegal. But maybe he doesn't. As a general rule, most people seem to have found that giving police new, broad sweeping powers is something they eventually come to regret.
"It may seem difficult at first, but everything is difficult at first."
Born in the US but returned to Mexico, in other words anchor baby. Though technically a US citizen, this guy was birthed here to cash in on our benefits, but spent his life in Mexico. Just imagine, if you were a Border Patrol officer, would you believe that someone that cannot speak English is an American citizen? Considering the proliferation of stolen/forged documents I would certainly have my doubts.
If you can't speak English, then you certainly can't read it, so how does one take a drivers test and get a drivers license if he can't even read the test. This is just another good reason why foreign nationals who come here in labor should not have American citizenship given to their offspring, especially when the mother is here illegally.
"First gallant South Carolina nobly made the stand."
Edge of Darkness
Actually if you are a legal alein then you have to carry your green card with you all of the time. My wife a legal US resident (applying for US citizenship now) and Canadain citizen has to carry her green card all of the time
From USCIS green card is issued to all permanent residents as proof that they are authorized to live and work in the United States. If you are a permanent resident age 18 or older, you are required to have a valid green card in your possession at all times. Current green cards are valid for 10 years, or 2 years in the case of a conditional resident, and must be renewed before the card expires.
A real man loves his wife, and places his family as the most important thing in life. Nothing has brought me more peace and content in life than simply being a good husband and father.
If you understand, things are just as they are... If you do not understand, things are just as they are....
- Zen Saying
Shockwave hit the nail on the head in his post--- there are three kinds of Americans he wrote: "A certain kind of American will read the above, nod his head and understand exactly what I'm talking about.
Another type will squint and feel uncomfortable: "You have done nothing wrong, so why not show ID?"
And yet another will think, "Dude, it's just ID - what's the problem?""
Hiibel was an horrendous ruling. The long term negative effects (which we are already experiencing in our daily lives) are going to fundamentally do great damage to freedom of ordinary people. And Hiibel is misunderstood as IT did not require the carrying of papers by US Citizens; only a willingness to state their name.
As for the folks who show ID in the Stop and Rob, ALL major credit card companies have provisions in their merchant agreements forbidding the merchant from asking for additional ID. And there are good reasons for that provision.
We all let Uncle get ahead of freedom when we didn't put up a fuss over airline demands for ID when security was still done by private companies and not TSA. We get screened, searched, they don't need ID and they don't need no-fly lists without means to get yourself removed. Without any process let alone due process.
Buy a ticket and get on board. That's how it worked with trains (Amtrack) and planes until relatively recently.
OBL never envisioned that his single act of violence would cause us to change from "the home of the brave" to the home of folks who give up freedom for a misperceived security advantage.
This isn't some form of pseudo nazi papers issue. You are required by law to have a valid DL to operate a motor vehicle. It's not a right it's a privilege. I have to have one on me and so do you. Are you saying that we should rally behind allowing illegal immigrants to drive without proper DL's and insurance because of "individual liberty"? Try that next time you are stopped for speeding or in an accident.
Did the system fail this particular individual? In as much as he is a citizen, yes it did. Not even going to argue validity of citizenship here. But if you are saying that we have an individual liberty to drive around without proper identification, we don't. Particularly if you break a law, like the guy in the article did when he was pulled for speeding. Why should we expect any less for immigrants? If we are going to wish for equal treatment, then lets have it. If you are operating a motor vehicle you have to have a valid DL and insurance. If you don't you go to court or jail. Sure let's treat them equally. By making them responsible when the law is broken, just like you and I.
How is allowing someone to break the law because they are not a citizen respecting individual liberty?
Last edited by chiefjason; September 16th, 2010 at 12:59 AM. Reason: spelling
I prefer to live dangerously free than safely caged!
"Our houses are protected by the good Lord and a gun. And you might meet 'em both if you show up here not welcome son." Josh Thompson "Way Out Here"
It's about as silly as the entire concept of the 2A specifying a preexisting right to be armed and to bear (carry) arms, yet that right effectively becomes a privilege with the permissions process, effectively legislated to the point it hardly exists in those states where it's non-issue (WI, IL) or might-possibly-issue-if-you're-rich-or-connected.
Silly, in a land of liberty populated with free citizens, eh? No politician has ever been able to satisfactorily explain to me why the privileged approach came to the carrying of arms or the driving of cars. I doubt any know or care, any longer, considering they're (at least from the time of being elected onward) a member of the "privileged" set.
Last edited by ccw9mm; September 17th, 2010 at 04:16 PM. Reason: grammar
Your best weapon is your brain. Don't leave home without it.
Thoughts: Justifiable self defense (A.O.J.).
Explain: How does disarming victims reduce the number of victims?
Reason over Force: Why the Gun is Civilization (Marko Kloos).
Proverbs 27:12 says: The prudent see danger and take refuge, but the simple keep going and suffer for it.
Certified Glock Armorer
NRA Life Member


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X3 config pleather?
View Single Post
Registered User
Location: South California
Join Date: Jul 2012
Posts: 79
Mein Auto: BMW
View My Garage
The only problem (which I experienced and eventually just gave up on) is that there is NO WAY in the US to get the desirable features of the premium package (dimming mirrors, ambient lighting, storage etc) with leatherette. BMW knows people want this. Therefore, they won't let you do it
So, you must get leather if you want the other features in the premium package.
For me, the dimming mirrors were a non-negotiable on a vehicle in this class. It's a shame BMW forces you to get leather for this. I guess you could retrofit some stuff but that's just annoying when you are paying this much for the vehicle.
There isn't anything inherently wrong with the leather... it just requires a tad more TLC. YMMV on wear and tear but if you take care of it, it should last you.
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Anna Edwards' Profile
Hello there, my name is Anna. I'm so happy you have found my little shop!
I am originally from Sweden but after many years of traveling and living in many different countries, I now simply consider myself a citizen of the earth I walk on.
So many things inspire me when I create my jewelry but tales of old and the rugged beauty of untouched nature are my greatest influences. I find beauty in the raw and the old, like hammer cut stones, vintage beads, rich patinas developed over time or created by artisan metal workers. My jewelry tends to be primarily rustic looking but a few playful fun pieces always find their way in too.
Aside from making jewelry, I have been drawing and painting since the day I was born and have just opened up a new shop with my original oil…
- Female
- Born on September 14
- Joined March 30, 2009
- Anna Edwards
- New Bloomfield, PA, United States


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Mea culpa, mea culpa, mea maxima culpa -.- I've been absent for so long I don't even know if someone's gonna remember me. Sorry for that, guys. Life can be a bitch.
Having said that, please enjoy the following piece and rest assured that I'm working again, so there's some to come ^^
Title: Rumpelstiltskin
Author: Enaty
Rating: T
Warning: slightly AU, spoiler for Volume 10
Disclaimer: Yes! More than just PSoH isn't mine! The fairy tale of course belongs to the Brothers Grimm. My pleasure, boys.
Claimer: I claim the idea for myself.
Author's Notes: After I did "Hansel & Gretel" for the Technical Incompatibility series, I was asked by Elvina whether I would write Snow White next. No, I won't. But I wrote this instead.
"No, Officer."
"Well, you've gotta have a name other than Count D! So just spit it and tell me!"
D turned and glared at the young man following him. "Why would you want to know, Officer? It's none of your business what my name is."
Leon grinned and stopped too, ignoring the exasperated looks that got him from other citizens of Chinatown. In fact, he even drew more attention by waving around a slip of paper. "Yeah, maybe, but, you know, I've gotta write your actual name on the warrant. And you yourself told me Count D's not your real name, so I'm trying to find out what it is."
D turned away from him with a look of exasperation on his face and continued making his way to the nearest bakery. Gods knew he needed sugar now.
"Okay, so is it a Chinese name? I don't know any Chinese names..."
"Which is such a blessing!" D snapped and briskly set his cup down to produce a hand fan from out of his sleeve, an action which made Leon roll his eyes.
"You are such a girl, really. Hey, I've got it! Desdemonda!"
A vein began to pulse on D's forehead. "Officer..." he gritted through his teeth, the warning clear in his voice. But Leon continued to fantasise; or perhaps he just liked annoying the hell out of D with this. There wasn't much else he could do to aggravate the pet shop owner, but his name had proven to be a sure way to make him lose him calm.
"Or perhaps it's not Desdemonda, but Daisy? A plant? Would fit, too, with you being all crazy for that green stuff... but you wouldn't be allowed to eat it if you were one yourself, would you?" He chuckled at his terrible joke, and then laughed even louder as D's teacup took flight. Luckily Leon had only been waiting for that to happen and managed to evade both the hand fan and his own teacup as he fled from the shop, calling "Princess Diana" back to D.
Leon was, of course, back the next day. "Hey, Dagobert," he said by way of greeting, and D's patience was finally worn thin.
"Who has ever told you that my name has to start with the letter D, I wonder," he snapped, glaring at the human. "It could start with any other letter; it might even start with a letter not common to the Latin alphabet, have you ever thought about that? Only because this shop's name is Count D does not necessarily mean my name starts with a D, too."
Leon relented easily to that suggestion. "Oh, I've thought 'bout that," he grinned and slumped down on the sofa. "But, you know, one's gotta start somewhere. I was going to start with E once I'm through with all D-names I can think of."
D's look was one of pure desperation.
Some weeks of silence followed that little event. Apparently Leon had grown tired of aggravating D with stupid names, and the kami was more thankful for that than he'd ever known he could be.
Then, out of the blue while they were having tea, Leon said: "Rumpelstiltskin."
D, standing at the tea-cart, froze, blinked a few times and then turned around, a threatening look on his face. Leon smiled at him innocently. "Wrong reaction, D. You should stomp one of your legs into the ground and then rip yourself in half."
D closed his eyes, took a deep breath, another, and a third. In fact, he didn't stop taking deep breaths until he'd counted to two hundred and thirty-five in his head, all the while Leon chuckled and seemed to be greatly amused.
Then he opened his eyes again and said, "Officer, although I have to admit that I am surprised by your knowledge of fairy tales, I have to tell you that if you ever – ever – start guessing my name again, I will permit T-chan to eat you."
Leon was still laughing quietly to himself, and his blue eyes sparkled merrily as he conceded. "Okay – Rumpelstiltskin."
And this time, the whole tea set was broken on his head.
However, the kami got used to Leon calling him thus; at least the name was one that wasn't completely unfitting, he consoled himself, although Leon of course couldn't know that. That didn't make it any less embarrassing when Leon caught up to him in the street, loudly calling for "Rumpelstiltskin", but he learned to deal with it, as he had learned to deal with all the other aggravating traits the officer had.
He never expected that this nickname would come in handy one day, but when Agent Howell handed him the warrant, proud and arrogant and self-assured, D couldn't help but laugh out loud. Although he tried to hide it behind his hand fan, the baffled, angry look on Howell's face, Leon's innocent-looking blue eyes – it was just too much.
"I am very sorry for having to disappoint you, Agent Howell," D said finally, wiping away a small tear and handing the warrant back to the FBI-agent. "But, as you surely know, you need a warrant with my real name on it. And, unfortunately, "Count Rumpelstiltskin D" is not amongst the choices."
Author's Notes: I don't have the slightest clue if a warrant really has to be issued with the true name of a person. But otherwise this wouldn't have worked, so just take it as I've written it, please ;-)


text:
Muscular Dystrophy and
the Cavalier King Charles Spaniel
- What It Is
- Symptoms
- Diagnosis
- DNA Testing
- Treatment
- Breeders' Responsibilities
- What You Can Do
- Research News
- Related Links
- Veterinary Resources
What It Is
Some cavalier King Charles spaniels suffer from a genetic form of muscular dsytrophy (MD) caused by a mutation of the dystrophin gene. Dystrophin is a protein that acts to stabilize skeletal and cardiac muscles and provides mechanical stability during the muscles' contractions. The dystrophin gene encodes that protein. The mutations in the dystrophin gene causes an absence of dystrophin in the muscles, which results in progressive degeneration of muscle fibres.
Muscular dystrophy is hereditary in the cavalier but is carried only by the X chromosome. Since males have only one X chromosome, they are more susceptible to inheriting MD because they need only one parent to pass the gene to them. For females to inherit the gene, both parents must carry it. Females are more often only carriers of the gene.
The genetic cause of muscular dystrophy in the CKCS was identified in a 2009 report by a research team at the UK Royal Veterinary College, led by Dr. Richard. J. Piercy and Gemma L. Walmsley. This study is on-going, with the cooperation of Dr. G. Diane Shelton at the Comparative Neuromuscular Laboratory. For example, in a December 2016 article, researchers applied "whole genome, next generation sequencing" (WGS) to evaluate a an MD-affected cavalier. WGS detected a deletion mutation in a DMD gene hotspot which is distinct from a previously identified mutation.
In a 2010 study, the same group of researchers have opined that all affected cavaliers may be related to a single founder. The extent of this disorder in the breed is not known. In a study examining 96 female CKCSs in the UK, there were no carriers identified. So, MD is suspected to be present at only a low level within the breed.
The type of MD in cavaliers is very similar to Duchenne muscular dystrophy (DMD) in humans. It reportedly afflicts one in 3,500 boys, and therefore it is one of the most common genetic disorders of children.
Since muscular dystrophy results in a lack of stability of the sketetal muscles, classic signs of MD are exercise intolerance, a stiff gait, and a lack of muscle mass. These symptoms usually are detectable at an early age, in some dogs, as young as six weeks to three months.
Other signs include: overall weakness, difficulty swallowing, an enlarged tongue, gagging when eating, breathing difficulties, muscle spasms, leg deformities, and tremors. The swallowing difficulty and gagging may result in aspiration pneumonia.
Symptoms in females usually are milder than in males.
Some mouth symptoms are similar to masticatory muscle myositis, another neuromuscular disease affecting the muscles used by the dog to chew – the jaw and temporal muscles.
Based upon the dog's symptoms, usually some sort of a neuromuscular disorder is suspected. The veterinarian should test the dog's reflexes, tongue, and jaw movement.
A blood test would reveal that serum creatine kinase (CK) is highly elevated. A muscle biopsy, under general anesthesia, should be taken for evaluation. Immunohistochemistry analysis of the biopsy is an essential test to determine the absence of dystrophin in the muscles.
Research laboratories are able to determine whether cavaliers carry the mutation of the gene. UK's Animal Health Trust, with the Royal Veterinary College have identified the genetic mutation. VetNostic Laboratories Labogen, Laboklin, and MyDogDNA offer a test based on the mutation.
DNA Testing
Research laboratories are able to determine whether cavaliers carry the mutation of the gene. UK's Animal Health Trust, with the Royal Veterinary College have identified the genetic mutation. VetNostic Laboratories Labogen, Laboklin, and MyDogDNA offer a test based on the mutation.
There is no cure for muscular dystrophy in the cavalier King Charles spaniel. Nor is there any medical therapy for maintenance of the disorder or to slow its progression. An exception to this could be anabolic steroid supplements or growth hormone treatments, which may temporarily slow the progression, but these drugs have potentially damaging side effects and may not be used long term.
Researchers are examining the efficacy of transplanting stem cells into dogs with muscular dystrophy. One such research project has reported increases in the dogs' level of dystrophin and regained muscle strength and mobility.
Other researchers are studying gene therapy to inactivate the offending gene (in this case the "exon 51") and restoring the expression of dystrophin. This is called antisense therapy. In a 2010 study, the researchers reported that "Antisense oligonucleotide-mediated skipping of exon 51 in cultured myoblasts from an affected [cavalier] dog restored the reading frame and protein expression". Translation: that is good, promising news.
For now, however, the prognosis is that affected cavaliers will not survive very long after severe symptoms appear. The lack of dystrophin eventually will begin to affect the contractibility of the dog's heart.
Breeders' Responsibilities
This form of muscular dystrophy is an hereditary disease passed by at least parent to its litters. Carriers can be identified. See DNA Testing, above.
Research News
December 2016: Whole genome, next generation sequencing detects new muscular dystrophy mutation in a cavalier. In a December 2016 article, a team of USA and Italian researchers (Nghiem PP, Bello L, Balog-Alvarez C, López SM, Bettis A, Barnett H, Hernandez B, Schatzberg SJ, Piercy RJ, Kornegay JN) applied "whole genome, next generation sequencing" (WGS) to evaluate a cavalier King Charles spaniel affected with muscular dystrophy (DMD). WGS detected a deletion mutation in a DMD gene hotspot which is distinct from a previously identified mutation.
August 2011: Cavaliers' DNA needed for muscular dystrophy test validation. VetNostic Laboratories needs 50 cavalier King Charles spaniels to contribute DNA samples to validate a test for dystrophin-deficient muscular dystrophy in the breed (CKCS-MD). Collection is performed using a cheek swab kit which consists of three swabs per dog. A self addressed, postage paid, return envelope will be provided. Once the lab receives the swabs, it will take about a week to obtain the results. Testing is free and result certificates will be provided. To obtain a collection kit along with instructions, please email Rob Mason email@example.com or call 877-255-9208. Website www.vetnostic.com
Related Links
Veterinary Resources
Efficient mapping of mendelian traits in dogs through genome-wide association. Elinor K Karlsson, Izabella Baranowska, Claire M Wade1, Nicolette H C Salmon Hillbertz, Michael C Zody, Nathan Anderson, Tara M Biagi, Nick Patterson, Gerli Rosengren Pielberg, Edward J Kulbokas III, Kenine E Comstock, Evan T Keller, Jill P Mesirov, Henrik von Euler, Olle Kämpe, Åke Hedhammar, Eric S Lander, Göran Andersson, Leif Andersson, Kerstin Lindblad-Toh. Nature Genetics. Sept. 2007;39:1321 - 1328. Quote: "With several hundred genetic diseases and an advantageous genome structure, dogs are ideal for mapping genes that cause disease. Here we report the development of a genotyping array with ~27,000 SNPs and show that genome-wide association mapping of mendelian traits in dog breeds can be achieved with only ~20 dogs. Specifically, we map two traits with mendelian inheritance: the major white spotting (S) locus and the hair ridge in Rhodesian ridgebacks. For both traits, we map the loci to discrete regions of <1 Mb. Fine-mapping of the S locus in two breeds refines the localization to a region of ~100 kb contained within the pigmentation-related gene MITF. Complete sequencing of the white and solid haplotypes identifies candidate regulatory mutations in the melanocyte-specific promoter of MITF. Our results show that genome-wide association mapping within dog breeds, followed by fine-mapping across multiple breeds, will be highly efficient and generally applicable to trait mapping, providing insights into canine and human health."
Muscular dystrophy in Cavalier King Charles spaniels. Piercy, Richard. J. and Walmsley, Gemma. Vet Rec. 2009 165 (2), p. 62. Quote: "We have recently identified the genetic cause of a form of muscular dystrophy in CKCS. The causative mutation is in the dystrophin gene and the X-linked disease is associated with weakness, muscle atrophy and exercise intolerance, detectable from a few months of age. Prominent signs in affected dogs are dysphagia [the symptom of difficulty in swallowing] and macroglossia (enlarged tongue)[tongue enlargement that leads to functional and cosmetic problems]. Serum creatine kinase is usually markedly elevated. Male dogs with the mutation [are] clinically affected and female dogs with the mutation are silent carriers. We are also keen to hear from veterinary surgeons who believe they may have seen an affected dog in their practice, in order to estimate the prevalence of this disease and limit its spread by genetic testing." Contact Dr. Piercy at the Royal Veterinary College's Comparative Neuromuscular Diseases Laboratory at firstname.lastname@example.com
Mutational Analysis of Dystrophin-deficient Muscular Dystrophy in Cavalier King Charles Spaniels. G. L. Walmsley, V. Arechavala-Gomeza, M. Fernandez-Fuente, N. Nagel, R. Stanley, K. Chandler, F. Muntoni, G. D. Shelton, and R.J. Piercy. J Vet Intern Med. 2009; 23(3):741 (ACVIM 27th Ann. Vet. Med. Forum Abstract Program: Abstract 189). Quote: "Canine dystrophin-deficient muscular dystrophy, analogous to Duchenne muscular dystrophy of humans, is a severe inherited degenerative disorder of striated muscle. This debilitating and ultimately fatal condition results in a progressive destruction of skeletal and cardiac muscle due to mutations in the gene encoding dystrophin, a structural protein that links the contractile apparatus to the sarcolemma. The disorder is seen in several canine breeds but the genetic cause has only been reported in the Golden Retriever, German Short-haired Pointer and Rottweiler. Here we present the findings of clinical, histopathological and molecular characterisation of this condition in Cavalier King Charles Spaniels. A 10 month old male neutered client-owned Cavalier King Charles Spaniel from the United Kingdom was presented with a chronic progressive history of lethargy, exercise intolerance and dysphagia. The dog was tetraparetic with poor skeletal muscle mass (body condition score 52/9), reduced spinal reflexes, macroglossia and restricted jaw movement. Investigations documented a marked elevation in creatine kinase activity (33,695U/l; 61–394U/l) and electromyography revealed spontaneous activity indicative of a primary muscle disorder (complex repetitive discharges and pseudomyotonia). Dystrophin deficient muscular dystrophy was diagnosed on the basis of skeletal muscle histopathology, immunohistochemistry and immunoblotting using monoclonal antibodies to the dystrophin rod and carboxy termini. Oligonucleotide primer pairs designed for RT-PCR to amplify overlapping regions of dystrophin cDNA identified, following sequencing, an exon deletion and a frame-shift not present in control cDNA, that is predicted to result in premature termination of the protein product. Sequencing the associated genomic DNA confirmed the causative (and novel) mutation. The ability of antisense oligonucleotide induced exon skipping to restore the reading frame was demonstrated in vitro in cultured myoblasts from the affected dog. Sequencing of amplified DNA from an additional Cavalier King Charles Spaniel with dystrophin-deficient muscular dystrophy from North America identified the same mutation. In conclusion, dystrophin deficient muscular dystrophy in the Cavalier King Charles Spaniel may provide an excellent model for Duchenne muscular dystrophy due to the potential application for trials of antisense oligonucleotide-mediated exon skipping – one of the more promising research directions for genetic therapy in this fatal disorder."
A Duchenne Muscular Dystrophy Gene Hot Spot Mutation in Dystrophin-Deficient Cavalier King Charles Spaniels Is Amenable to Exon 51 Skipping. Gemma L. Walmsley, Virginia Arechavala-Gomeza, Marta Fernandez-Fuente, Margaret M. Burke, Nicole Nagel, Angela Holder, Rachael Stanley, Kate Chandler, Stanley L. Marks, Francesco Muntoni, G. Diane Shelton, Richard J. Piercy. J. Plos One. January 2010; 5(1):e8647. Quote: Background: Duchenne muscular dystrophy (DMD), which afflicts 1 in 3500 boys, is one of the most common genetic disorders of children. This fatal degenerative condition is caused by an absence or deficiency of dystrophin in striated muscle. Most affected patients have inherited or spontaneous deletions in the dystrophin gene that disrupt the reading frame resulting in unstable truncated products. For these patients, restoration of the reading frame via antisense oligonucleotide-mediated exon skipping is a promising therapeutic approach. The major DMD deletion "hot spot" is found between exons 45 and 53, and skipping exon 51 in particular is predicted to ameliorate the dystrophic phenotype in the greatest number of patients. Currently the mdx mouse is the most widely used animal model of DMD, although its mild phenotype limits its suitability in clinical trials. The Golden Retriever muscular dystrophy (GRMD) model has a severe phenotype, but due to its large size, is expensive to use. Both these models have mutations in regions of the dystrophin gene distant from the commonly mutated DMD "hot spot". Methodology/Principal Findings: Here we describe the severe phenotype, histopathological findings, and molecular analysis of Cavalier King Charles Spaniels with dystrophin-deficient muscular dystrophy (CKCS-MD). ... Here we describe a spontaneous canine model of DMD in the Cavalier King Charles Spaniel (CKCS), a toy breed, specifically bred for its small size (5– 8 kg) and amiable temperament. In this manuscript we report the clinical, pathological and molecular characterisation of the model and investigate the suitability of exon skipping-mediated therapy. In this study we sought to determine the genetic basis of dystrophin-deficient muscular dystrophy in 3 CKCS dogs. The novel splice site mutation of the dystrophin gene that we have identified in these affected dogs from the UK and the USA occurs in a region that is most commonly mutated in humans, and gives rise to a single exon deletion (exon 50) in the dystrophin mRNA. ... The index case was a 10-month-old male neutered client-owned CKCS (see photo) from the United Kingdom that was presented to the Royal Veterinary College for investigation of a generalised neuromuscular disorder. The dog had been in the owners' possession from 2 months of age and the puppy had always been of small stature, poorly muscled and exercise intolerant; the main presenting complaint, however, was a 3 month progressive history of dysphagia. ... Two additional young male CKCS from the USA were also diagnosed with dystrophin deficient muscular dystrophy (dogs 2 and 3). Dog 2 was a 6-month-old intact male that was presented to a general veterinarian because of dysphagia and decreased range of jaw motion, first noted by the owner at 2 months of age. A marked elevation in serum CK activity (64,918 U/L) was measured. Dog 3 was an 8-month-old neutered male that was presented to the Veterinary Teaching Hospital, University of California, Davis, with a history of gagging when eating and drinking since acquired as a 6-week-old puppy, combined with exercise intolerance. On physical examination, restricted jaw mobility and macroglossia were noted. The serum CK activity was markedly elevated (58,508 U/L). Thoracic radiography revealed a normal shape and size to the cardiac silhouette and echocardiography revealed very mild mitral and tricuspid regurgitation (believed to be clinically irrelevant) and normal left ventricular function. Electromyography revealed spontaneous repetitive discharges and pseudomyotonia in all muscles examined. ... The dogs harbour a missense mutation in the 5′ donor splice site of exon 50 that results in deletion of exon 50 in mRNA transcripts and a predicted premature truncation of the translated protein. Antisense oligonucleotide-mediated skipping of exon 51 in cultured myoblasts from an affected dog restored the reading frame and protein expression. Conclusions/Significance: Given the small size of the breed, the amiable temperament and the nature of the mutation, we propose that CKCS-MD is a valuable new model for clinical trials of antisense oligonucleotide-induced exon skipping and other therapeutic approaches for DMD. ... Further work, ideally utilizing a colony of CKCS-MD dogs, would enable comprehensive phenotypic characterization of various organ systems, including the heart. Furthermore, if the cardiac muscle changes are found to be comparatively mild in this model, it would provide an opportunity to evaluate factors contributing to this phenotype.
Mammalian Models of Duchenne Muscular Dystrophy: Pathological Characteristics and Therapeutic Applications. Akinori Nakamura and Shin'ichi Takeda. J.Biomed.Biotechnol.; Jan 2011; 2011:184393. Quote: "5.3. Cavalier King Charles Spaniels with Muscular Dystrophy (CKCS-MD) -- 5.3.1. Pathological Characteristics -- Very recently, it was reported that another dystrophic dog, the Cavalier King Charles Spaniel with dystrophin-deficient muscular dystrophy (CKCS-MD) has a severe phenotype. This canine model has a missense mutation in the 5′ donor splice site of exon 50 resulting in deletion of exon 50 in mRNA transcripts and a predicted premature truncation of the translated protein. 5.3.2. Therapeutic Applications -- The therapeutic strategy of exon skipping in the GRMD or CXMDJ models provides a significant advantage in clinical trials, but also has the unfavorable characteristic that the disease-causing mutation does not include the region of the DMD gene that is most commonly mutated in human DMD patients. About 60% of DMD patients harbor deletions in exons 45–55 of the DMD gene [55, 56]. As described previously, the exon 51 skipping technique, which is being employed in clinical trials [30, 31], is applicable to DMD patients which have deletions of exon 50, exon 52, exons 48–50, or exons 49-50, among others, and is feasible for more patients than other exon skipping strategies [27, 57, 58]. AO-mediated skipping of exon 51 in cultured myoblasts of the CKCS-MD dog restored the reading frame and protein expression [17]. This observation suggests that the use of this canine model would be valuable in the preclinical trials of therapy based upon exon 51 skipping."
Genetic Connection: A Guide to Health Problems in Purebred Dogs, Second Edition. Lowell Ackerman. July 2011; AAHA Press; pg 127. Quote: "Cavalier King Charles spaniels ...are susceptible to an X-linked myopathy similar to Duchenne-type muscular dystrophy in humans. ... The defect has been characterized in the Cavalier King Charles spaniel ... and so it is likely that other genetic tests will eventually become available."
Canine models of Duchenne muscular dystrophy and their use in therapeutic strategies. Joe N. Kornegay, Janet R. Bogan, Daniel J. Bogan, Martin K. Childers, Juan Li, Peter Nghiem, David A. Detwiler, C. Aaron Larsen, Robert W. Grange, Ratna K. Bhavaraju-Sanka, Sandra Tou, Bruce P. Keene, James F. Howard Jr., Jiahui Wang, Zheng Fan, Scott J. Schatzberg, Martin A. Styner, Kevin M. Flanigan, Xiao Xiao, Eric P. Hoffman. Mamm. Genome. Jan. 2012;23(1):85-108. Quote: "The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied in 7 dogs from birth to 12-14 months and in 18 dogs at varying intervals from birth to 8 weeks. One affected male was studied from age 3.5 to 6 years, and all pups were descendants of this dog. A lethal neonatal form was recognized in some pups. In the more typical form, clinical signs of stunting, weakness and gait abnormalities were evident by 6-9 weeks and were progressive, leading to marked muscle atrophy, fibrosis and contractures by 6 months. Serum CK levels were markedly elevated, such that affected pups could be identified by 1 week. CK values increased until 6-8 weeks, then plateaued at approx. 100 times normal. Affected females and beagle-cross dogs were less severely affected than large breed-cross dogs. In the 2 adult dogs with cardiac insufficiency CK levels had decreased to 5-15 times normal. These studies show that CXMD and Duchenne muscular dystrophy have striking phenotypic as well as genotypic similarities. In addition, these studies of CXMD suggest that in females and in smaller dogs the same genetic defect results in a less severe clinical disease."
Inhibition of CD26/DPP-IV enhances donor muscle cell engraftment and stimulates sustained donor cell proliferation. Maura H Parker, Carol Loretz, Ashlee E Tyler, Lauren Snider, Rainer Storb, Stephen J Tapscott. Skelet Muscle. 2012; 2: 4. Published online 2012 February 16. doi: 10.1186/2044-5040-2-4 Quote: "Background: Transplantation of myogenic stem cells possesses great potential for long-term repair of dystrophic muscle. In murine-to-murine transplantation experiments, CXCR4 expression marks a population of adult murine satellite cells with robust engraftment potential in mdx mice, and CXCR4-positive murine muscle-derived SP cells home more effectively to dystrophic muscle after intra-arterial delivery in mdx5cv mice. Together, these data suggest that CXCR4 plays an important role in donor cell engraftment. Therefore, we sought to translate these results to a clinically relevant canine-to-canine allogeneic transplant model for Duchenne muscular dystrophy (DMD) and determine if CXCR4 is important for donor cell engraftment. Methods: In this study, we used a canine-to-murine xenotransplantation model to quantitatively compare canine muscle cell engraftment, and test the most effective cell population and modulating factor in a canine model of DMD using allogeneic transplantation experiments. Results: We show that CXCR4 expressing cells are important for donor muscle cell engraftment, yet FACS sorted CXCR4-positive cells display decreased engraftment efficiency. However, diprotin A, a positive modulator of CXCR4-SDF-1 binding, significantly enhanced engraftment and stimulated sustained proliferation of donor cells in vivo. Furthermore, the canine-to-murine xenotransplantation model accurately predicted results in canine-to-canine muscle cell transplantation. Conclusions: Therefore, these results establish the efficacy of diprotin A in stimulating muscle cell engraftment, and highlight the pre-clinical utility of a xenotransplantation model in assessing the relative efficacy of muscle stem cell populations."
Cardiac structure and function in female carriers of a canine model of Duchenne muscular dystrophy. A.M. Kane, T.C. DeFrancesco, M.C. Boyle, D.E. Malarkey, J.W. Ritchey, C.E. Atkins, J.M. Cullen, J.N. Kornegay, B.W. Keene. Research in Vet. Sci. Dec. 2012. Quote: "This investigation tested the hypothesis that carriers of golden retriever muscular dystrophy (GRMD), a genetically homologous condition of Duchenne muscular dystrophy (DMD), have quantifiable abnormalities in myocardial function, structure, or cardiac rhythm. Eleven GRMD carriers and four matched controls had cardiac evaluations and postmortem examinations. 24-h ECG Holter monitoring disclosed ventricular ectopy in 10 of 11 carriers and 2 of 4 controls. Conventional echocardiography failed to demonstrate significant differences between carriers and controls in systolic function. All carriers had multifocal, minimal to marked myofiber necrosis, fibrosis, mineralization, inflammation, and/or fatty change in their hearts. Immunohistochemistry revealed a mosaic dystrophin deficiency in scattered cardiac myofibers in all carriers. No controls had cardiac histologic lesions; all had uniform dystrophin staining. Despite cardiac mosaic dystrophin expression and degenerative cardiac lesions, GRMD carriers at up to 3 years of age could not be distinguished statistically from normal controls by echocardiography or 24-h Holter monitoring."
Duchenne muscular dystrophy gene therapy in the canine model. Dongsheng Duan. Human Gene Therapy Clinical Development. January 2015. doi:10.1089/hum.2015.006. Quote: "Duchenne muscular dystrophy (DMD) is an X-linked lethal muscle disease caused by dystrophin deficiency. Gene therapy has significantly improved the outcome of dystrophin-deficient mice. Yet, clinical translation has not resulted in the expected benefits in human patients. This translational gap is largely due to the insufficient modeling of DMD in mice. Specifically, mice lacking dystrophin show minimum dystrophic symptoms and they do not respond to the gene therapy vector in the same way as human patients do. Further, the size of a mouse is hundredfolds smaller than a boy, making it impossible to scale-up gene therapy in a mouse model. None of these limitations exist in the canine DMD (cDMD) model. For this reason, cDMD dogs have been considered a highly valuable platform to test experimental DMD gene therapy. Over the last three decades, a variety of gene therapy approaches have been evaluated in cDMD dogs using a number of non-viral and viral vectors. These studies have provided critical insight for the development of an effective gene therapy protocol in human patients. This review discusses the history, current status and future directions of the DMD gene therapy in the canine model."
Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy. Joe W. McGreevy, Chady H. Hakim, Mark A. McIntosh, Dongsheng Duan. Disease Models & Mechanisms. March 2015;8(3):195–213. Quote: "Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disorder. It is caused by loss-of-function mutations in the dystrophin gene. Currently, there is no cure. A highly promising therapeutic strategy is to replace or repair the defective dystrophin gene by gene therapy. Numerous animal models of DMD have been developed over the last 30 years, ranging from invertebrate to large mammalian models. mdx mice are the most commonly employed models in DMD research and have been used to lay the groundwork for DMD gene therapy. After ~30 years of development, the field has reached the stage at which the results in mdx mice can be validated and scaled-up in symptomatic large animals. The canine DMD (cDMD) model will be excellent for these studies. In this article, we review the animal models for DMD, the pros and cons of each model system, and the history and progress of preclinical DMD gene therapy research in the animal models. We also discuss the current and emerging challenges in this field and ways to address these challenges using animal models, in particular cDMD dogs. ... Dystrophin gene mutations have been mapped in nine cDMD breeds (although only four mutations have been published in peer-reviewed research articles). Point mutations have been found in the Cavalier King Charles spaniel muscular dystrophy dogs (CKCS-MD, intron 50), golden retriever muscular dystrophy dogs (GRMD, intron 6) and Rottweiler muscular dystrophy dogs (exon 52). Deletion mutations have been found in three breeds, including a small four-nucleotide deletion in exon 65 (Cocker spaniel), an exon 8–29 deletion in the Tibetan terrier, and a whole-gene deletion in the German shorthaired pointer. "
Whole genome sequencing reveals a 7 base-pair deletion in DMD exon 42 in a dog with muscular dystrophy. Nghiem PP, Bello L, Balog-Alvarez C, López SM, Bettis A, Barnett H, Hernandez B, Schatzberg SJ, Piercy RJ, Kornegay JN. Mamm Genome. December 2016. Quote: Dystrophin is a key cytoskeletal protein coded by the Duchenne muscular dystrophy (DMD) gene located on the X-chromosome. Truncating mutations in the DMD gene cause loss of dystrophin and the classical DMD clinical syndrome. Spontaneous DMD gene mutations and associated phenotypes occur in several other species. The mdx mouse model and the golden retriever muscular dystrophy (GRMD) canine model have been used extensively to study DMD disease pathogenesis and show efficacy and side effects of putative treatments. Certain DMD gene mutations in high-risk, the so-called hot spot areas can be particularly helpful in modeling molecular therapies. Identification of specific mutations has been greatly enhanced by new genomic methods. Whole genome, next generation sequencing (WGS) has been recently used to define DMD patient mutations, but has not been used in dystrophic dogs. A dystrophin-deficient Cavalier King Charles Spaniel (CKCS) dog was evaluated at the functional, histopathological, biochemical, and molecular level. The affected dog's phenotype was compared to the previously reported canine dystrophinopathies. WGS was then used to detect a 7 base pair deletion in DMD exon 42 (c.6051-6057delTCTCAAT mRNA), predicting a frameshift in gene transcription and truncation of dystrophin protein translation. The deletion was confirmed with conventional PCR and Sanger sequencing. This mutation is in a secondary DMD gene hotspot area distinct from the one identified earlier at the 5' donor splice site of intron 50 in the CKCS breed.


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Starting 0-6 stinks.
Yeah, there's a headline for you.
You don't need to tell Perrish Cox that, because he knows all too well. His
Call that the Anti-Yogi-ism
Six games in that seemed like a pipe-dream, but now with two wins in a row under their belt, Cox says his team does have a silver lining in the horizon. "We need to win this last game and we can make it into the playoffs," Perrish said. "We got our first win a couple of weeks ago, but the big one was just this last weekend, when we beat Midway."
Midway high school was 5-2 on the season before their game with the "Trojans" and they were undefeated in district play. The eye-popping 41-7 win over the "Panthers" not only kept University's post-season hopes alive, but gave them a lot of hope for the immediate future. "I can't honestly tell you why we played as well as we did against Midway even though I knew we could play like that before the season even started," Perrish said. "I guess that win last week boosted everyone's confidence and everyone came out and got it done."
Going into his final year Cox had a lot of aspirations as to just what he thought his team could do. He had his individual goals as well, but he knew that based on last year's standout performance, where he tallied 9 interceptions playing DB, along with 27 tackles, including a sack, plus the 47 receptions, 670 yards and 12 touchdowns he had playing wideout, he knew he was a marked man this year.
"I was getting a lot of that at points during last year, so I figured it was going to be double and triple-teams this year," Cox said of seeing not only cornerbacks, but linebackers and safeties covering him in games.
"It was funny about Midway, though, because when everyone is doing all this crazy stuff to try and limit what I was doing, Midway actually tried to cover me one-on-one."
"That was just stupid."
Cox wasn't about to squander the advantage, catching two touchdown passes, one a 45 yard reception, where Cox leapt over the defender at the goal line and as he was fading back, coming to the ground for the score.
It was an attention-grabbing catch for an attention grabbing playmaker that was happy to work off a season's worth of frustration, where he's seen multiple coverage when he's on offense and almost nobody around him when he's on defense. As you can see, it's hard to blame the competition.
If you man cover him, he goes ballistic, something he's been glad to do, having only 4 other touchdowns on the season from the fact that nobody else made Midway's mistake. If you try to go toward Cox when he's on defense, he's got six interceptions to say thanks for the gesture, one of those taken back for a touchdown. And if you try kicking to him, he's got two touchdowns off of kick returns this year.
Cox finds himself frustrated, but complimented at the same time. "You get the idea they don't want to have anything to do with you after awhile," Perrish said of how little offenses throw his way or how much coverage he sees when he's on offense ."You'd like them just to challenge you straight up, but I guess they've seen what can happen when they do that."
"It's ok, because I am used to it. It has just made me raise my game to take advantage of the opportunities I have."
On the recruiting front Cox has a
lot more opportunities than what opponents are giving him on the field. He's got
offers from all of his favorites (LSU,
With the late date for the visit to see the Cowboys, Cox will more than likely be taking his decision all the way to signing day. Up until then, he said that he's intentionally keeping things as vanilla as he can regarding teams that he likes. "I am trying to keep everything real open right now," Cox said. "I have seen a couple of places, both of them I liked a lot, but that's not all the schools I want to see."
"Once I see them all, I will have a good idea of what is going to work for me the best and where will be the place I really feel I fit in."
Perrish is being recruited to
play either side as you might expect. He'll opt to play defense first, but is
open to playing wide receiver as well. Of the schools he's visited,
Now, Cox just has bigger fish to fry, this one named Ellison. As in high school, Cox's team hoping to make it three in a row, so they can make what just three weeks ago probably seemed impossible; a berth in the first round of the post season.
Considering where they came from to where they are now, Cox is realistic, but he even admits that he thinks his team is finally starting to believe. "If you believe you can win, you can win," he said. "I knew we had the team to do it and now we are just going out there and playing like we plan on winning the game."
"We're going to do that this week and we'll see how it all turns out. I am really confident, though. I think we have a run coming."


text:
21 November 2007,
Price realised EUR 1,875
Estimate EUR 1,500 - EUR 2,000
Pearl Perlmutter (American, b. 1915)
Sitting man
bronze with a dark brown patina
24 x 27 cm. (incl. the bronze base)
Conceived in 1946 in an edition of circa 4
New York +1 212 636 2000
London +44 (0)20 7839 9060
Hong Kong +852 2760 1766
Shanghai +86 21 6355 1766
Special Notice
Artist's Resale Right ("Droit de Suite"). Artist's Resale Right Regulations 2006 apply to this lot, the buyer agrees to pay us an amount equal to the resale royalty provided for in those Regulations, and we undertake to the buyer to pay such amount to the artist's collection agent.Christie's charges a premium to the buyer on the Hammer Price of each lot sold at the following rates: 29.75% of the Hammer Price of each lot up to and including €5,000, plus 23.8% of the Hammer Price between €5,001 and €400,000, plus 14.28% of any amount in excess of €400,001. Buyer's premium is calculated on the basis of each lot individually.
Saleroom Notice
Please note that the edition of this work is 8 plus 2 AP
Arnhem, Gemeente Museum Arnhem, Pearl Perlmutter beeldhouwster, October - November 1988 (the terracotta version ill.)


text:
Follow-up appointments and veterinary client responsibility...to their pets
Last week's post on fat dogs and legal remedies occasioned a comment on the hypocrisy involved in prosecuting owners for negligence and cruelty when prosecuting veterinarians for similar lapses happens so infrequently.
I'd argue both scenarios are as rare as your average male calico cat. (For the record, that's about one in 3,000.)
OK so my stats may be a little fuzzy here, but given that vets comprise a tiny percentage of the potential animal cruelty offenders, it seems not unreasonable that veterinary prosecutions for cruelty are so infrequently seen. When it does occur, the media tends to take it on to ensure swift and decisive action against the individual.
Consider the case of the Tennessee shelter vet charged after video footage of his intra-cardiac euthanasias surfaced. Though I'd argue this vet deserves a professional defrocking (not forthcoming, I'm afraid), the penalties are nonetheless serious and their full extent still remain to be seen.
In the case mentioned in the comment to Fat dogs…, a client failed to seek follow-up care for his pet after a procedure. I surmise he was charged with some form of negligence.
Here in Miami this happens all the time—sans prosecution. A whopping 10% of my clients are unlikely to return for their follow-up appointments in a timely fashion.
So you know, recheck removal appointments after procedures involve more than just a couple of swipes of the suture-removal scissors. The goal is to have a look at the pet to ensure proper healing has taken place.
Sure, some of my clients take out their own pets' sutures and some pets never even get sutures in the first place. I nonetheless require they come back for a look-see (usually covered by the cost of the initial procedure so there's no fee for the follow-up).
This failure to follow-up situation is far worse when a bandage is involved. A broken leg, an ear hematoma, a wound of some sort…all commonly require frequent rechecks to ensure healing is progressing as planned. When owners fail to show the consequences can be truly disastrous. And too often the vet is held responsible—by the owner.
Take last week's case belonging to a neighbor vet. She and I dished over a client's failure to come back for a follow-up three months after the specialized procedure. The client was called numerous times. It was all documented in the records.
Finally, the groomer (yes, the groomer) called to yell at this vet for leaving the pet in such a state. He accused her of negligence and cruelty for not properly attending to the dog. Luckily, he also supplied the client's work number through which she was finally able to contact the client.
Without batting an eye, the client agreed to come back to the hospital, though she was very angry over the state of her pet's disrepair. Needless to say this doc was expecting the worst, wondering what kind of legal hassles she'd have to deal with—likely the result of the client's failure to comply with medical orders.
Now, no one likes to see a pet in pain. But I daresay this vet breathed a huge sigh of relief when she saw that her surgery had healed beautifully. It was the opposite side that was suffering this time. The owner had confused one side's symptoms for the other.
This owner had preferred to rail over her vet's cruelty and negligence to anyone who would listen…when she couldn't tell her left from her right—and couldn't be bothered to make her way back for a recheck despite the obvious "complication."
What if it had been the same side? What if a timely re-examination could've made all the difference? To what degree is an owner's negligence worthy of legal redress?
Surprisingly, surgery-gone-wrong-after-home-care-gone-awry cases mostly end in veterinary malpractice suits—not in cruelty charges against owners.
I know, it's not exactly fair to attack a client by slapping them with a charge of cruelty for failing to arrive for a re-examination visit (or not giving the antibiotics or failing to use the provided E-collar, etc.). After all, some clients simply suffer from garden-variety ignorance. And as they say, you can't legislate common sense.
We vets have to be compassionate towards owners who don't always completely understand us, no matter how obvious we believe we've been in detailing their pets' needs and the consequences of not seeing to them. But there's a limit, isn't there?
It's important for all of us to keep in mind that there are always two sides to every story. There's often a pretty good reason why the client failed to show or the wound got weepy. It's not always someone's fault.
Sure, egregious examples exist but I like to think of them the same way I consider my calicos: overwhelmingly female.


text:
U.S. Senator John Boozman, a Republican from Arkansas, talked about the upcoming State of the Union address, criticized some of President Obama's recent decisions, and discussed other election year issues, in his monthly visit with KASU. Click on "Listen" for the full interview.
Boozman discusses election year issues on KASU
By MSMITH • Jan 24, 2012


text:
Walkthrough Part 24 - Drill-X's Big Rig 2/2 for Skylanders GiantsHome / Playstation 3 /
This page contains Skylanders Giants Video Walkthroughs for Playstation 3 called "Walkthrough Part 24 - Drill-X's Big Rig 2/2" and has been posted or updated on Dec 28, 2012 by Blitzwinger.
- Developer: Toys For Bob
- Publisher: Activision
- Genre: Action Platformer
- Release: Oct 21, 2012
- ESRB: Everyone 10+
Walkthrough Part 24 - Drill-X's Big Rig 2/2
Watch this step-by-step Video Walkthrough, which may help and guide you through each and every level part of this game.
Please Submit a Problem for any incomplete, non-working or fake cheat listed above.
If you know other secrets, hints, glitches or level guides, then please Submit your Cheats and share your insights with other players.
From: Dal
From: skeeter
Stuck in this game? Set a specific, clearly defined question and let other gamers answer your question or view answers to previously asked questions. If you think you are an expert then please try to help others with their questions.
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