
ghadakam dii

manushyaril "sii. eph. dii" jeane encode cheiyunna oru proteinaan factor dii (, "sii3 proaaktivettar convertase", "properdine factor dii estaresu", "factor dii (compliment)", "compliment factor dii", "sii. eph. dii", "adipsin"). bii gadakathe pilarthunna pooraka samvidaanathinte badal pooraka pathayil ghadakam dii ulppedunnu.
pravartthanam.
eee jeane encode cheytha protein adiposaittukal rakthapravaahathilekk sravikkunna serin proteesukalude tripsin kudumbatthile amgamaanu. pakarchavyaadhikale humoural adichamarthunnathile pankinu peruketta badal pooraka paathayude oru gadakamaanu encode cheytha protein. avasaanamaayi, encode cheytha protein kozhuppil uyarnna thothilulla aavishkaaramundu, ithu rogaprathirodha samvidaana jeevashaasthrathil adipose tissuek oru punk nirddeshikkunnu.
factor dii ennathu oru serin proteasaanu, ithu kozhuppu koshangalil triglyceride adinjukoodunnathinu glucose gathaagathathe uthejippikkukayum lipolysis thadayukayum cheiyunnu.
klinikkal praadhaanyam.
ponnatthadi rogikalil dii ghadakam kurayunnu. eee kuravu uyarnna pravartthanamo prathirodhamo moolamaakaam, pakshe krithyamaaya kaaranam poornnamaayum ariyilla.
ghadana.
serin proteesukalude chemotrypsin kudumbatthile ellah amgangalkkum valare samanamaaya gadanakalundu. ellah saahacharyangalilum, ghadakam dii ulppede, rund aantipaarallal β-barrell domainukalundu, oro baralilum aarr β-straandukal adangiyirikkunnu, ellah ensaimukalilum ore typology undu. dii gadakavum chemotrypsin kudumbatthile mattu serin proteasukalum thammilulla nattellu gadanayile pradhana vyathyasam dvitheeya gadanaaparamaaya gadakangale bandhippikkunna uparithala looppukalilaan. crotripsin kudumbatthil saadhaaranayaayi kaanapedunna pradhana kaattalatik, substrate-binding avashishtangalude vyathyastha roopeekaranangal ghadakam dii pradarshippikkunnu. eee savisheshathakal soochippikkunnathu oru punarkrameekaranatthiloode roopeekarana maattangalkk prerippikkunnathuvare ghadakam dii yude kaattalatik pravartthanam nirodhichirikkunnu ennaanu.
pravartthana samvidaanam.
rakthathilum tissuevilum sajeevamayathum ennaal svayam niyantrithavumaaya roopeekaranathil adangiyirikkunna oru serin proteasaanu factor dii. dii gadakathinte ariyappedunna eaka swaabhaavika substrate ghadakam bii aanu, koodaathe bii gadakathile aarji 234-lies 235 skisile bondinte pilarpp bii sakalangalaaya bii, bii. bii ennee rund gadakangalkku kaaranamaakunnu. bii gadakathile kathrika bondinte villal sambhavikkunnathin munbu, sii3bibi complexes roopeekarikkunnathinu munbu bii ghadakam aadyam sii3biyumaayi bandhippikkanam. sii3bibi complexile ghadakam biyude eee anuroopa maattam ghadakam biye ghadakam diyude binding saittilekk poruthappedaan anuvadikkunnuvennu nirddeshikkappedunnu.
factor dii yude kaattalitik triad aspa 102, hiss 57, serr195 enniva chernnathaanu. factor diyude mattu pradhana gadakangal oru svayam-inhibittari lupineyum (amino assid avashishtangal 212 muthal 218 vare) noonn-canonical konformationil hiss 57 side cheyineyum sthirappeduthunna oru aspa189-aarji 218 salt bridjaanu. svayam-prathirodha lupe athinte thadassappeduthiya roopathil, ghadakam bii-yude ghadakam dii-yilekkulla praveshanam thadayunnu. ghadakam dii yude svayam niyantritha roopeekaranathe sii3bibi complexes sameepikkumbol, sii3bibi ghadakam dii yill uppu paalathe sthaanabhrashtamaakkukayum ghadakam bii yude aarji234 num ghadakam dii yude asp189 num idayil oru puthiya uppu paalathinu kaaranamaakukayum cheiyunnu. factor dii salt bridginte sthaanachalanathinte falamaayi svayam-prathirodha looppinte punarvinyaasavum sajeevamaaya saitt histidin side cheyinte bhramanavum sambhavikkunnu, ithu factor dii-yude canonical roopam srishtikkunnu. bii gadakathile kathrika bandhatthinte villal thudarnnu sambhavikkunnu, ithu khandam bii. eiii. vidukayum sii3-convertase enna badal paathayaya sii3 bbb roopappedukayum cheiyunnu.
niyanthranam.
karalum adipposaittukalum chernnaanu dii ghadakam samanvayippikkunnathu, randaamathethu pradhana uravidamaanu. sravikkunna dii gadakathinte proo-phoom masp-3 upayogichu pilartthi shareerathil sanjarikkunna sajeeva kramam roopappeduthunnu. dii ghadakam valare uyarnna substrate prathyekatha nilanirthunnu, athinte falamaayi shareerathil ariyappedunna prakrithidatha inhibittarukal illa. ennirunnaalum, dii gadakathinte bhooribhaagavum kaattalatik cytilekkulla substrate axus parimithappedutthunna svayam niyantritha roopathilaanu. dii gadakathinu 23.5 kiloo dii aanava bhaaramund, aarogyamulla manushyaril ithu 1.8 milligram/lii rakthathinte saandrathayil kaanapedunnu. ghadakam prathidinam ekadesham 1.33 milligram/kilogram enna synthesis nirakk ullathinaal, veendum agiranam cheythathinushesham proximal tubuleukalile kaattabolisathinu sesham dii gadakathinte bhooribhaagavum vrukkayiloode illaathaakkappedunnu. manikkooril 60 sathamaanam enna uyarnna fraakshanal metaboolik nirakkaanu nettu ephakt. vrukkayude saadhaarana pravartthanamulla rogikalil moothrathil dii ghadakam kandethanaayilla. ennirunnaalum, vrukkasambandhamaaya rogamulla rogikalil, ghadakam dii uyarnna thalatthil kandethi. dii ghadakam kuranja nilavaaratthil polum pravartthikkaan badal paathaykk kazhiyum, dii gadakathinte nilavaaratthile kurravukal apoorvamaanu.
rogangalil punk.
chromosome 19 leeii dii jeenil oru stopp kodoon (tagg) upayogichu oru serin kodoon (serr 42 prosess cheyyaatha methionin roopathilulla ghadakam dii) maattisthaapikkunnathinu oru pooyint mutation ghadakam dii kuravinu kaaranamaayi rekhappeduthiyittundu. dii gadakathinte kuravu bacteria anubaadhakalilekkulla, prathyekichu niseriya anubaadhakalilekkulla saadhyatha vardhippikkum. dii kuravinte anantharaavakaasha reethi autosomal resussive aanu, oru alleelil maathram mutation ulla vyakthikalkk veendum anubaadhaykkulla athe saadhyatha anubhavappedanamennilla. veendum anubaadhayulla oru rogiyil, shudheekaricha ghadakam dii avatharippichukondu avasthayil poornnamaaya purogathi labhichu.
amithamaya pooraka sajeevamaakkal ulla rogangalil paroxismal noctornal haemoglobinuria (pnh) ulppedunnu, koodaathe factor diyude inhibittarukalkku pnhinte chikilsayil upayogamundakaam. factor dii yude cheria molecule inhibittarukal pnhinte chikilsaykkaayi vikasippichukondirikku, koodaathe oru cheria molecule inhibittaraaya aachu-4471, eculisumabinoppam samyojippikkumbol factor dii inhibishanaayi randaam ghatta klinikkal trialil vaagdaanam cheythittundu. factor dii inhibittarukal upayogichu chikilsikkunna rogikalkku dii gadakathinte kurravulla rogikaleppole veendum anubadha undaakunnath ozhivakkaan anubaadhaykkethire prathirodha kuthivayppu nalkanam.

