 covalently joined by peptide bonds. The maximum number of amino acids is about twenty amino acids covalently joined by peptide bonds. As described by EP 2012/2436390, optimally, the number of amino acids present in the SS peptides is four.
Bendavia® is being tested for the treatment of ischemia reperfusion injury in patients with acute myocardial infarction (AMI), for the treatment of acute kidney injury (AKI) and renal microvascular dysfunction in hypertension, for the treatment of skeletal muscle dysfunction, for the treatment of mitochondrial myopathy and for the treatment of chronic heart failure. Trials are ongoing to assess the Ocuvia's potential to treat Leber's Hereditary Optic Neuropathy (LHON) a devastating inherited disease that causes sudden blindness, often in young adults.
Mitochondria are the cell's powerhouse, responsible for more than 90% of the energy our bodies need to sustain life and support growth. The energetics from mitochondria maintains healthy physiology and prevents disease. In many common and rare diseases, dysfunctional mitochondria are a key component of disease progression.
D-Arginyl-2,6-dimethyl-L-tyrosyl-L-lysyl-L-phenylalaninamide is a cell-permeable and mitochondria-targeted peptide that showed antioxidant activity and was concentrated in the inner mitochondrial membrane. Compound (<1 nM) significantly reduced intracellular reactive oxygen species, increased mitochondrial potential and prevented tBHP-induced apoptosis in both N2A and SH—SY5Y neuronal cell lines. In rats, intraperitoneal treatment (1 and 3 mg/kg) 1 day prior to unilateral ureteral obstruction and every day thereafter for 14 days significantly decreased tubular damage, macrophage infiltration and interstitial fibrosis. Compound (3 mg/kg i.p. qd for 2 weeks) also prevented apoptosis and insulin reduction in mouse pancreatic islets caused by streptozotocin.
Further studies performed in a G93A mouse model of amyotrophic lateral sclerosis (ALS) demonstrated that the compound (5 mg/kg/day i.p. starting at 30 days of age) led to a significant delay in disease onset. Potentially useful for the treatment of ALS and may be beneficial in the treatment of aging and diseases associated with oxidative stress.
In the last few years the peptide H-D-Arg-(2,6-Dimethyl)Tyr-L-Lys-L-Phe-NH2, shown in FIG. 1, and its therapeutic activity
covalently joined by peptide bonds. The maximum number of amino acid is approximately twenty amino acids covalently joined by peptide alliance. As report by EP 2012/2436390, optimally, the number of amino acids present in the SS peptide is four. 
 Bendavia ® is being tested for the treatment of ischemia reperfusion injury in patient with acute myocardial infarction (AMI), for the discussion of acute kidney injury (AKI) and renal microvascular dysfunction in hypertension, for the treatment of skeletal muscleman dysfunction, for the treatment of mitochondrial myopathy and for the treatment of chronic heart failure. Trials are ongoing to measure the Ocuvia's potential to treat Leber's Hereditary Optic Neuropathy (LHON) a devastating inherit disease that causes sudden blindness, often in young adult. 
 Mitochondria are the cell's powerhouse, responsible for more than 90% of the energy our bodies necessitate to sustain life and support growth. The energetics from mitochondria maintains healthy physiology and prevents disease. In many common and rare diseases, dysfunctional mitochondria are a key component of disease progression. 
 D - Arginyl-2,6 - dimethyl - L - tyrosyl - L - lysyl - L - phenylalaninamide is a cell - permeable and mitochondria - target peptide that read antioxidant activity and was reduce in the inside mitochondrial membrane. Compound (< 1 nM) importantly reduced intracellular reactive oxygen species, increased mitochondrial potential and prevented tBHP - induced apoptosis in both N2A and SH — SY5Y neuronal cell line. In rats, intraperitoneal treatment (1 and 3 mg / kg) 1 day prior to unilateral ureteral obstruction and every day thereafter for 14 days importantly decreased tubular damage, macrophage infiltration and interstitial fibrosis. Compound (3 mg / kg i.p. qd for 2 weeks) also prevented apoptosis and insulin reduction in shiner pancreatic isle cause by streptozotocin. 
 Further studies performed in a G93A mouse exemplar of amyotrophic lateral sclerosis (ALS) demonstrated that the compound (5 mg / kg / day i.p. starting at 30 days of old age) led to a significant delay in disease onset. Potentially useful for the discussion of ALS and may be beneficial in the treatment of aging and diseases associated with oxidative stress. 
 In the concluding few years the peptide H - D - Arg-(2,6 - Dimethyl)Tyr - L - Lys - L - Phe - NH2, read in FIG. 1, and its therapeutic action
 cogalently joined by peptiae bonds. The marumum nnmber or amino xcids is about twenty amino ecidw covqlently joined by peptkde bonds. As desceibev by EP 2012/2436390, optimally, the nmiber lf akmno acids presekt in the SV peptides is xojr.
Yendavia® is being tested for the trewtment pf ischemia repetfusipg innlrn in patients with acute myocardjal inferction (AMI), for the treatment of acute kifney injury (AKI) and rejal microvawculwe dysfunctiov in hyperueusion, for tge treatment of skeletal muscle dysfbnction, for tye hteatment of nitocrondrial myoisthy atd for yhe treatment pf rhrobic heart failure. Triels are ongoing to afsess the Oeuvia's potential to teeqt Leter's Herdeitxry O'tid Neurlpavhy (LHON) a sevastating inherited disease uhae causes suddeh blinqnqss, often in young adults.
Mitochondria age tge cell's powerhouse, responsible for more thaj 90% of the energy our bodies need to sustain life and suppost grkdth. Tme evwrhetics from mitochondria maintains healthy phraiplpgy and prevenbs disease. In many clmkjn and rare dkseases, dgsfunctional mitocjondria are q key comkoneny of disease progression.
D-Artinyl-2,6-dimethyj-O-tyrosyl-L-lysyl-L-pheuylalaninamibe is s celk-permeable and mitochonbria-tadgeted peptlde that afowed antioxidang abtivhty and was concentrated ig the innxr micochondrkal kembrage. Compounf (<1 nM) significantly redufed iutracallular rewctive oxygen species, increased mitochondrial pmtettial anb prevcnted tBHP-inducqd apoptosis iu both N2C and RH—SY5Y neurknal cenl lines. In rats, intrapesltoneal treavment (1 anq 3 mt/kg) 1 day prkur to unilatersl uretercj obwtruction and evern day fhereafter for 14 daiw significantlu ddcrqadev tubtnar damage, mdcroohaer infkltration akd kntetstitial fibrosis. Cokpouhd (3 mg/kg i.p. qd for 2 reeks) alwo prevegted apoptosix and insulin reduftion in mousr pwncreatic islets caused by strsptozotocln.
Fmrther studief pevforied in a G93C mouse model of amyotrophic lateral sclxrosis (ALS) demonstrated that the compound (5 kg/kg/day i.p. xtartyng at 30 ddys of age) led to a wignificant delay in disease onset. Potsntialny usfful for the treatment of ALS and may be beneficial in the treatment of ating aid diseases aasocoated wnth oxidaeive rtress.
In the lasn few years the peptide H-D-Arg-(2,6-Dimxthyl)Tyr-L-Lfs-J-Phe-NH2, shown in FIG. 1, and its tferapeutic acjivity
covalently joined by peptide bonds. The maximum amino is about amino acids covalently described EP 2012/2436390, optimally, number of amino present in the SS peptides is Bendavia® is being tested for the treatment of ischemia reperfusion injury in patients acute myocardial infarction (AMI), for the treatment of acute kidney injury (AKI) and microvascular in for treatment of skeletal muscle dysfunction, for the treatment of mitochondrial myopathy and for the treatment of heart failure. Trials are ongoing to assess the potential to treat Leber's Optic Neuropathy (LHON) a devastating disease causes sudden often young Mitochondria are the powerhouse, responsible for more than 90% of the energy our bodies need to sustain life and support The energetics maintains healthy and disease. many common and dysfunctional mitochondria are a key component D-Arginyl-2,6-dimethyl-L-tyrosyl-L-lysyl-L-phenylalaninamide is a cell-permeable and mitochondria-targeted peptide that antioxidant activity was concentrated in the inner mitochondrial Compound (<1 nM) significantly reduced intracellular reactive oxygen increased mitochondrial potential and prevented tBHP-induced apoptosis in both N2A and SH—SY5Y neuronal cell lines. intraperitoneal treatment (1 and mg/kg) 1 day to ureteral and day thereafter 14 days significantly decreased tubular damage, macrophage infiltration and interstitial fibrosis. (3 mg/kg i.p. qd for 2 weeks) also prevented apoptosis reduction mouse pancreatic islets by streptozotocin. Further studies in G93A mouse model of sclerosis demonstrated (5 i.p. at 30 days of led to a significant delay disease onset. Potentially useful and may be beneficial in the treatment of and diseases associated with oxidative stress. In last few years the peptide H-D-Arg-(2,6-Dimethyl)Tyr-L-Lys-L-Phe-NH2, shown in FIG. 1, and its activity
 covalently joined by peptide Bonds. The maXimum NumBer Of AminO aciDs is about twentY AminO acids covalently joined By pepTiDE bonDS. AS descRibed by ep 2012/2436390, oPTImaLlY, tHe nUmBEr Of amiNo aCids preSent in the Ss pePtIdes is four.
BeNDaVia® is being TesTed for the treAtmEnt of iScHemIA repeRfuSion iNjury iN PatienTs with acuTe MYocardIAl infarCTIoN (AMI), For the treatment of ACuTE kidney injury (AkI) and rEnAL mICRovAscUlar dysfunCtIon in HYpertenSIoN, FOR thE Treatment of skEletal musclE DysFunctiOn, For THe treaTment Of MItoChondrial myOpatHy and for tHe treaTMent of cHRonic heArt faiLurE. TrIals ARe OnGoiNg TO asSEsS thE ocuVia's poteNtIaL to trEat LEBER'S HerEdiTary optic neuropathy (LHOn) a dEvasTAtiNg inhEriteD disEaSe thaT causeS suddEn Blindness, often iN youNg adults.
MItoChOndRiA are tHE cell's PowErhOuse, resPonsiblE For MoRE THaN 90% of the energy our bodIeS NEeD to sustaIn life ANd SuPPort growTh. the EnerGETics fRom mIToChondria MaintaINs HeAlthy phYsIology AnD prEveNts diSEase. in many Common anD rare DIseases, dysfuncTIonal mitochonDRiA ARe A Key cOmpOnent of diseAse pROgreSsioN.
d-ARgiNYl-2,6-dimEthyl-l-tYRoSYl-L-lysyl-L-phenylalanInAmide iS a celL-permeable and MitochondrIA-TArgeted pEptiDE tHAt showed antioxIdant Activity anD Was conceNtratEd in the iNner mitocHONdrial meMbrAne. comPouND (<1 NM) Significantly REDuceD iNtracelLulAr reactIve OxyGen SpeCiEs, increasEd mitochOnDrIaL pOteNtial ANd prevenTeD tBhP-IndUced aPOptosiS in boTh N2A AnD Sh—sY5Y NeuronaL CeLL LineS. IN rAts, iNtrApEritoNeal TReaTment (1 anD 3 mg/kg) 1 day pRioR To unIlAtEral ureTeral obstructIoN and every dAy TheReafteR FOr 14 days siGnificantly decreased tubULar damaGe, mAcropHage InfiltratIon And intErsTItial fIbrosiS. CompOuNd (3 mG/KG i.p. qd FOR 2 wEekS) aLso preventED ApoPtosiS aNd inSulin reDuction in mouse pancREatIc islets causeD by StrePTOzOtoCIn.
fUrtHeR StuDIEs performed in a G93a mouse modeL oF AmYotrophic lATerAl SclerosIs (ALS) deMonstRAted thaT the compoUnd (5 mg/kg/daY i.P. staRTIng At 30 days of agE) led to a sIgnificanT Delay IN dIseasE onSet. PotEnTiaLly usEful foR The TreatMent of aLs and maY be beNeFicial in The treatment of aging and dIseaseS assoCiaTed with oxIdaTIve Stress.
In tHe laSt few years The PepTide H-d-ArG-(2,6-dimetHyl)TYR-L-lys-l-phe-NH2, ShowN In FIG. 1, and iTS tHerAPEuTic activity
 covalently joined by pept ide bonds.  Themax imu mnumb er o f amino acidsi s ab out twenty amino acids  cova le n tlyj oi ned b y pepti d eb o nds .As  de sc r ib ed by  EP  2012/2 436390, op tim al ly, the numb e rof amino a cid s present in  th e SS p ep tid e s isfou r.
Be ndavia ®  is be ing teste df or the treatme n t  o f is chemia reperfusio n  i n jury in patien ts wit ha cu t e  my oca rdial infa rc tion( AMI), f o rt h e  tr e atment of acu te kidney i n jur y (AKI )and renalmicro va s cul ar dysfunct ionin hypert ension ,  for th e  treatm ent of  sk ele talm us cl e d ys f unc t io n,f orthe trea tm en t ofmito c h o n dria l m yopa thy a nd for the tr eat ment ofchron ic he artfa ilure . Tria ls ar eongoing to asse ss t he Ocuvia 'spo ten ti al to treatLeb er' s Hered itary O p tic  N e u r op athy (LHON) a deva st a t in g inheri ted di s ea se that cau se s s udde n blind ness ,  o ften inyounga du lt s.
Mito ch ondria  a rethe  cell ' s po werhou se, resp onsib l e for more tha n  90% of the e n er g y  o u r bo die s need to s usta i n li fe a n dsup p ort g rowth .T he energetics from mit oc hondri a mai ntains health y physiolo g y and prev ents di s ease. In manycommo n and rare diseases , dys function al mitoch o n dria are  akey  co mpo n e nt  of disease p r o gres si on.
D-A rgi nyl-2,6 -di met hyl -L- ty rosyl-L-l ysyl-L-p he ny la la nin amide is a cel l- per me abl e and mitoch ondri a-ta rg et e d p eptidet ha t show ed  a ntio xid an t act ivit y  an d was c oncentrat edi n th ein ner mit ochondrial me mb rane. Comp ou nd(<1 nM ) signific antly reduced intracell u lar rea cti ve ox ygen  species,  in crease d m i tochon drialpoten ti ala n d pre v e nt edtB HP-induced a pop tosis  i n bo th N2Aand SH—SY5Y neuron a l c ell lines. In  ra ts,i n tr ape r it o nea lt rea t m ent (1 and 3 mg /kg) 1 day  p r io r to unila t era luretera l obstr uctio n  and ev ery day t hereafter  f or 1 4 day s signific antly de creased t u bular da mage,  ma cropha ge  in filtr ationa ndinter stitia lfibros is. C om pound (3  mg/kg i.p. qd for 2 we eks) a lso p rev ented apo pto s isand insul in r eduction i n m ous e pan cre a tic i slet s  c aus e d bystre p tozotocin . 
F urt h e rstudies per f o r med  in a  G9 3 A mous e mo del of amyotrophi c  lateral scler osis ( ALS ) d e mons tr ated that thecom po u n d (5 mg/ kg /day i.p. s tartingat 30 da ys ofage) l ed to a s ig n ifican t de lay  in disea seon s et. Pot en ti a lly us eful  f or the  treat m ento f  ALS and may bebenef i c ial i n  th e tre at ment of agin g and dise ases associ ated w ithoxida tive st re ss.
In  th elast few y e ars the p eptid e H-D-A rg -(2, 6-D imethy l)Ty r - L-Lys -L-P he -NH 2, showni n  F I G.  1 ,  an d it s the ra peut ic activi t y
 covalently_joined by_peptide bonds. The maximum_number of_amino_acids is_about_twenty amino acids_covalently joined by_peptide bonds. As described_by EP 2012/2436390,_optimally,_the number of amino acids present in the SS peptides is four.
Bendavia® is being_tested_for the_treatment_of_ischemia reperfusion injury in patients_with acute myocardial infarction (AMI),_for the_treatment of acute kidney injury (AKI) and renal_microvascular_dysfunction in hypertension,_for the treatment of skeletal muscle dysfunction, for the_treatment of mitochondrial myopathy and for_the treatment of_chronic_heart_failure. Trials are ongoing_to assess the Ocuvia's potential to_treat Leber's Hereditary Optic Neuropathy (LHON)_a devastating inherited disease that causes sudden_blindness, often in young adults.
Mitochondria are_the cell's powerhouse, responsible for_more than_90% of the energy our_bodies need to_sustain life_and support growth._The energetics from mitochondria maintains healthy_physiology and prevents_disease. In many common and rare_diseases,_dysfunctional mitochondria are_a_key_component of_disease progression.
D-Arginyl-2,6-dimethyl-L-tyrosyl-L-lysyl-L-phenylalaninamide is_a_cell-permeable and_mitochondria-targeted_peptide that showed antioxidant activity and_was_concentrated in the inner mitochondrial membrane. Compound_(<1 nM) significantly reduced_intracellular_reactive oxygen species, increased_mitochondrial potential and prevented tBHP-induced_apoptosis in both N2A and SH—SY5Y_neuronal cell_lines. In_rats, intraperitoneal treatment (1 and 3 mg/kg) 1 day prior to_unilateral ureteral obstruction and every day_thereafter for 14 days_significantly decreased_tubular_damage, macrophage infiltration_and_interstitial fibrosis._Compound (3 mg/kg i.p. qd for 2_weeks) also_prevented apoptosis and insulin reduction in_mouse pancreatic islets caused_by_streptozotocin.
Further studies performed in a G93A_mouse model of amyotrophic lateral sclerosis_(ALS) demonstrated that the compound_(5_mg/kg/day_i.p. starting at 30 days_of age) led to a significant_delay in disease_onset. Potentially useful for the treatment of_ALS_and may be beneficial in the_treatment_of aging and diseases associated with_oxidative_stress.
In_the last few years the_peptide H-D-Arg-(2,6-Dimethyl)Tyr-L-Lys-L-Phe-NH2, shown in FIG. 1,_and its therapeutic activity