KEGG ORTHOLOGY: K18186

ORTHOLOGY: K18186

Entry

K18186                      KO

Symbol

PET100

Name

protein PET100, animal type

Disease

H01368

Cytochrome c oxidase (COX) deficiency

Brite

KEGG Orthology (KO) [BR:ko00001]
09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K18186  PET100; protein PET100, animal type
Mitochondrial biogenesis [BR:ko03029]
Mitochondrial quality control factors
  Mitochondrial respiratory chain complex assembly factors
   Complex-IV assembly factors
    K18186  PET100; protein PET100, animal type

Genes

HSA:	100131801(PET100)

PTR:	100610902 101057124

PPS:	100983712 100993393

GGO:

PON:

PPYG:

NLE:

HMH:

SSYN:

MCC:	708563(PET100)

MCF:

101865429

MTHB:

126942879

MNI:	105484419(PET100)

CSAB:

103233807(PET100)

CATY:

105589144(PET100)

PANU:

101017597

TGE:	112612524(PET100) 112636688

MLEU:

105541139(PET100)

RRO:

104679482

RBB:	108534540(PET100)

TFN:

117076083

PTEH:

111522612

CANG:

105512906(PET100)

CJC:

100405707

SBQ:	101033543(PET100)

CIMI:

108283672

ANAN:

105706056

CSYR:

103252242(PET100)

MMUR:

105885639(PET100)

LCAT:

123635332

PCOQ:

105807864(PET100)

OGA:	100966556(PET100)

MMU:	100503890(Pet100)

MCAL:

110300346

RNO:	688786(Pet100)

MCOC:

116068835

ANU:

117698329

ASYL:

127672749

MUN:	110542360 132650559

CGE:

MAUA:

PROB:

PLEU:

MORG:

AAMP:

NGI:	103743300(Pet100)

HGL:	101701469(Pet100)

CPOC:

100719776(Pet100)

CCAN:

109690963(Pet100)

DORD:

105998762(Pet100)

DSP:

PLOP:

NCAR:

MMMA:

ITI:

OCU:

OPI:

TUP:	102481995(PET100)

GVR:	103596855(PET100)

CFA:	100685574 611528

CLUD:

112666437 112668968

VVP:	112909733(PET100) 112917431

VLG:	121480187 121495838

NPO:

AML:

UMR:

UAH:

UAR:

ELK:

LLV:

MPUF:

101670710(PET100)

MNP:

132009682

MLK:

131823446

NVS:

122909788

ORO:	101386536(PET100)

EJU:	114200843(PET100)

ZCA:

113917165

MLX:	118005887 118017433

NSU:	110582697 110593948

LWW:	102727639 102747728

FCA:	101096955(PET100)

PYU:

121013912

PCOO:

112852637(PET100)

PBG:

PVIV:

LRUF:

LGF:

AJU:

PTG:	102966587(PET100)

PPAD:

109254188(PET100)

PUC:

125928982

PLEZ:

122213894

HHV:

120244821

BTA:	132342162 616224(PET100)

BOM:	102274068(PET100) 102283396

BIU:	109561645(PET100) 109568285

BBUB:

102410992

BBIS:

104979775(PET100)

CHX:	102170221(PET100) 108635042

OAS:

101122597

BTAX:

128051519

CSUM:

138085486(PET100)

ODA:

120863908

CCAD:

122440362

MREE:

136166541(PET100)

OVR:	110121575 110152509(PET100)

MBEZ:

129558494

SSC:	100037981(PET100)

CFR:

102521289

CBAI:

105078276(PET100)

CDK:

105087563

VPC:

102543059

BACU:

103015345(PET100)

BMUS:

118891840

LVE:	103088744(PET100)

OOR:

LALB:

DLE:

PCAD:

PSIU:

NASI:

112400119(PET100)

ECB:	100066498(PET100)

EPZ:	103540060(PET100)

EAI:

106844515

MYB:	102254868(PET100)

MYD:	102768398(PET100)

MMYO:

118657672

MLF:	102422868(PET100)

MDT:

132234616

MYUM:

139010003(PET100)

PKL:

118725795

EFUS:

103294995

MNA:	107540636(PET100)

DRO:	112296414(PET100)

SHON:

AJM:

PDIC:

PHAS:

MMF:

PPAM:

HAI:	109396109(PET100)

RFQ:

117037803

PALE:

102895257(PET100)

PGIG:

120599016

PVP:	105307690(PET100)

RAY:

107519923

MJV:

108399690

TOD:

119239821

SARA:

101556911(PET100)

SFUM:

130022437

SETR:

126020439

LAV:	100660921(PET100)

TMU:

101345290

ETF:

101659906

DNM:	101423440(PET100)

MDO:	100026950(PET100)

GAS:

123232302

SHR:

100930332

AFZ:

127545334

PCW:	110203363(PET100)

TVP:

118851137

PBRV:

138165402(PET100)

OAA:

TACU:

CJO:

TGU:

LSR:	110483029(PET100)

PMOA:

120508282

OTC:

121339184

PRUF:

121364414

ATRC:

139585694(PET100)

PHI:	102101810(PET100)

ACOE:

138100153(PET100)

ZLE:

135441444

ACHL:

103797950(PET100)

CCIC:

134055516

AGEN:

126037510

FGA:	104075373(PET100)

CBOY:

140645148(PET100)

TGT:	104567747(PET100)

ASN:	102379964(PET100)

PSS:	102454131(PET100)

CMY:

CCAY:

CPIC:

TST:

CABI:

MRV:

ACS:	100567767(pet100)

ASAO:

132768490

PVT:	110080434(PET100)

SUND:

121922163

PBI:	103051796(PET100)

PMUR:

CTIG:

PGUT:

APRI:

PTEX:

113433594(PET100)

NSS:	113418202(PET100)

VKO:

PMUA:

PRAF:

ZVI:

HCG:

GJA:	107111611(PET100)

STOW:

125428376

EMC:	129327416 129328362

XLA:	108711654(pet100.L)

XTR:	100493963(pet100)

NPR:	108800580(PET100)

RTEM:

120933195

BBUF:

120986266

BGAR:

122929431

PWL:	138292027(PET100)

MUO:	115459440 115466174

GSH:

117350580

DRE:	100334510(pet100)

SRX:	107711253 107757350

SANH:

107670483 107672445

SGH:	107563330 107587949

CCAR:

109049013 109049035

CAUA:

113041854 113049411 113081380 113095441

CGIB:

PTET:

LROH:

RKG:

MAMB:

CIDE:

CERY:

137013001(pet100)

MASI:

127450838

TROS:

130561711

TDW:

130425636

MANU:

129430801(pet100)

PDAB:

135735953 135744820

IPU:

108257119

IFU:

128600835

PHYP:

113546199(pet100)

SMEO:

TFD:

TVC:

CGAR:

NGRA:

TRN:

AMEX:

CMAO:

EEE:	113581859(pet100)

CHAR:

105905734 122133183

SPIL:

134077330

TRU:

101061557

TNG:	GSTEN00007301G001

LCO:	104918569(pet100)

NCC:	104952494(pet100)

TBEN:

117493432

CGOB:

115011708

PGEO:

117450698(pet100)

GACU:

EMAC:

ELY:

EFO:

PLEP:

SLUC:

ECRA:

ESP:

PFLV:

GAT:

PPUG:

AFB:

CLUM:

PSWI:

CANA:

137786977(pet100)

CVE:	137878619(pet100)

MSAM:

119896530

SCHU:

122869110

CUD:

121529130

SPUL:

138603148(pet100)

LMIX:

132954556

ALAT:

119014814

ASTR:

137609540(pet100)

MZE:	101484897(pet100)

ONL:	100708333(pet100)

OAU:

116317665

OLA:	101158698(pet100)

OML:

112146316

CSAI:

133421630

XMA:	102235900(pet100)

XCO:	114160549(pet100)

XHE:

116736132

PRET:

103468754(pet100)

PFOR:

103152025(pet100)

PLAI:

106946697(pet100)

PMEI:

106915642(pet100)

GAF:

122821576

PPRL:

129363241

CVG:	107093430(pet100)

CTUL:

119785345

GMU:

124865258

NFU:	107378330(pet100)

KMR:

108241282

ALIM:

106529539(pet100)

NWH:

119421736

AOCE:

111580745

BFRE:

138623469(pet100)

MCEP:

124997416

CSEM:

103392866(pet100)

POV:	109634106(pet100)

SSEN:

SSOE:

HHIP:

HSP:

PPLT:

SMAU:

LCF:

SDU:	111227118(pet100)

SLAL:

111653716(pet100)

XGL:

120807187

HCQ:	109507906(pet100)

SSCV:

125983936(pet100)

SBIA:

133514186

PEE:

133415173

PTAO:

133465440

BPEC:

110169684(pet100)

ENY:	140357667(pet100)

MALB:

109965909(pet100)

BSPL:

SJO:

SSCO:

GMH:

SASA:

STRU:

OTW:

OMY:	110485140 110537983

OGO:

123999018

ONE:

115110101

OKI:	109893178 109897250

OKE:

118384344

OMM:

135555898

OCLA:

139421040 139423545

SALP:

111980646(pet100)

SNH:

CCLU:

ELS:

OEP:

SFM:

PKI:	111849247(pet100)

AANG:

118216457

AROT:

135243172

CCOG:

133114661

LOC:	102685804(pet100)

PSPA:

121304614

ARUT:

117401976 117965570

PSEX:

120539977

LCM:	102357768(PET100)

RTP:

CPLA:

HOC:

LERI:

PMRN:

LRJ:

BFO:

BBEL:

SPU:	100889189 115928858

LPIC:

AJJ:

APLC:

ARUN:

AAMU:

AFIL:

AJC:

DME:	Dmel_CG14483(CG14483)

DER:

6546747

DSE:

6609636

DSI:	Dsimw501_GD25461(Dsim_GD25461)

DYA:	Dyak_GE13982

DAN:

DSR:

DPO:

DPE:

DMN:	108159782 117192887

DWI:

6637912

DGR:

6559266

DMO:	Dmoj_GI20787

DAZ:

DNV:

DHE:

DVI:

CCAT:

BOD:

BDR:

RZE:

AOQ:

ALUD:

TDA:	119682724 119683327

MDE:

SCAC:

LCQ:

LSQ:

CVJ:

GFS:

ECOE:

CLON:

HIS:

ACOZ:

AARA:

AMER:

ASTE:

AMAP:

AFUN:

AMOU:

AMRS:

AALI:

ACOS:

AAG:	110678982 5567496

AALB:

109410759

ASUA:

134226917

CQU:	CpipJ_CPIJ016904 CpipJ_CPIJ019629

CPII:

CBRI:

CNS:

BCOO:

LLL:

PPAP:

BVK:

BPYO:

FVI:

CCAL:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

COBS:

TLN:

HST:

DQU:

CFO:

FEX:

PMEC:

LHU:

OBO:

PCF:

PFUC:

VPS:

NVI:

TPRE:

MDL:

FAS:

CCIN:

DSM:

NPT:

NFB:

NLO:

NVG:

AROA:

TCA:

TMOL:

DPA:

ESIM:

EFOR:

AGRG:

ATD:

AGB:

LDC:

DVV:	114327188 114333917

NVL:	108558092 108566586

APLN:

OTU:

BMOR:

BMAN:

MSEX:

DPL:	KGM_202065

BANY:

MJU:

NIQ:

VCD:

MCIX:

PMAC:

BPHI:

PRAP:

PBX:

PNAP:

ZCE:

CCRC:

LSIN:

AAGE:

HAW:

HZE:

TNL:

SLIU:

OFU:

ONU:

GMW:

PGW:

LGLY:

CSTB:

CFEL:

CCRN:

API:

DNX:

AGS:

RMD:

ACOO:

DVT:

BTAB:

CLEC:

HHAL:

HVI:

MQU:

BROR:

IEL:

HAMR:

DPX:	DAPPUDRAFT_230548

MNZ:

MRJ:

PORN:

HAZT:

EAF:

TCF:

PPOI:

CEL:	CELE_Y53F4B.14(Y53F4B.14)

CBR:

CBG_20908

CRQ:	GCK72_006649

NAI:	NECAME_10277

PCAN:

LSAX:

BGT:

GAE:

OSN:

LJP:

LLON:

NVE:

EPA:

ATEN:

AMIL:

MCAZ:

MFOO:

PDAM:

SPIS:

DGT:

XEN:

RES:

AQU:

Reference

PMID:24462369

Authors

Lim SC, Smith KR, Stroud DA, Compton AG, Tucker EJ, Dasvarma A, Gandolfo LC, Marum JE, McKenzie M, Peters HL, Mowat D, Procopis PG, Wilcken B, Christodoulou J, Brown GK, Ryan MT, Bahlo M, Thorburn DR

Title

A founder mutation in PET100 causes isolated complex IV deficiency in Lebanese individuals with Leigh syndrome.

Journal

Am J Hum Genet 94:209-22 (2014)
DOI:10.1016/j.ajhg.2013.12.015

Sequence

[hsa:100131801]

LinkDB

DBGET integrated database retrieval system