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(PET100)

PPS:	100983712 100993393

GGO:

101148063

PON:	100456216(PET100)

NLE:

100601835

HMH:

116478359

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

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

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:

OCU:

OPI:

TUP:	102481995(PET100)

GVR:	103596855(PET100)

CFA:	100685574 611528

CLUD:

112666437 112668968

VVP:	112909733(PET100) 112917431

VLG:	121480187 121495838

NPO:

129521379

AML:

100466097

UMR:

103681743

UAH:	113242930(PET100)

UAR:

123777557

ELK:

111161869

LLV:

125085633

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:

122488634

PVIV:

125160210

LRUF:

124524635

PTG:	102966587(PET100)

PPAD:

109254188(PET100)

PUC:

125928982

AJU:

106970821

HHV:

120244821

BTA:	132342162 616224(PET100)

BOM:	102274068(PET100) 102283396

BIU:	109561645(PET100) 109568285

BBUB:

102410992

BBIS:

104979775(PET100)

CHX:	102170221(PET100) 108635042

OAS:

BTAX:

ODA:

CCAD:

MBEZ:

SSC:	100037981(PET100)

CFR:

102521289

CBAI:

105078276(PET100)

CDK:

105087563

VPC:

102543059

BACU:

103015345(PET100)

BMUS:

118891840

LVE:	103088744(PET100)

OOR:

DLE:

PCAD:

PSIU:

NASI:

112400119(PET100)

ECB:	100066498(PET100)

EPZ:	103540060(PET100) 103546000

EAI:

106844515

MYB:	102254868(PET100)

MYD:	102768398(PET100)

MMYO:

118657672

MLF:	102422868(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(PET100)

MJV:

108399690

TOD:

119239821

SARA:

101556911(PET100)

SETR:

126020439

LAV:	100660921(PET100)

TMU:

101345290

ETF:

101659906

DNM:	101423440(PET100)

MDO:	100026950(PET100)

GAS:

123232302

SHR:

100930332

AFZ:

127545334

PCW:	110203363(PET100)

OAA:

CJO:

TGU:

LSR:

PMOA:

OTC:

PRUF:

PHI:	102101810(PET100)

ACHL:

103797950(PET100)

AGEN:

126037510

FGA:	104075373(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

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:

MASI:

TROS:

TDW:

MANU:

IPU:

IFU:

PHYP:

113546199(pet100)

SMEO:

124376085

TFD:

113648096

TVC:

132864140

AMEX:

103037501(pet100)

CMAO:

118806129

EEE:	113581859(pet100)

CHAR:

105905734 122133183

TRU:

101061557

TNG:	GSTEN00007301G001

LCO:	104918569(pet100)

NCC:	104952494(pet100)

TBEN:

CGOB:

PGEO:

GACU:

EMAC:

ELY:

EFO:

PLEP:

SLUC:

ECRA:

ESP:

PFLV:

GAT:

PPUG:

AFB:

CLUM:

PSWI:

MSAM:

SCHU:

CUD:

ALAT:

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(pet100)

MCEP:

124997416

CSEM:

103392866(pet100)

POV:	109634106(pet100)

SSEN:

HHIP:

HSP:

SMAU:

LCF:

SDU:	111227118(pet100)

SLAL:

111653716(pet100)

XGL:

120807187

HCQ:	109507906(pet100)

SSCV:

SBIA:

PEE:

PTAO:

BPEC:

110169684(pet100)

MALB:

109965909(pet100)

BSPL:

SJO:

SASA:

STRU:

OTW:

OMY:	110485140 110537983

OGO:

123999018

ONE:

115110101

OKI:	109893178 109897250

OKE:

118384344

SALP:

111980646(pet100)

SNH:

CCLU:

ELS:

SFM:

PKI:	111849247(pet100)

AANG:

118216457

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:

129271530 129271531

APLC:

110977406

AJC:

117110000

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:

TDA:	119682724 119683327

MDE:

SCAC:

LCQ:

LSQ:

GFS:

ECOE:

CLON:

HIS:

ACOZ:

AARA:

AMER:

ASTE:

AFUN:

AMOU:

AALI:

AAG:	110678982 5567496

AALB:

109410759

CQU:	CpipJ_CPIJ016904 CpipJ_CPIJ019629

CPII:

CNS:

BCOO:

BVK:

BTER:

BPYO:

FVI:

CCAL:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

HST:

DQU:

CFO:

FEX:

LHU:

OBO:

PCF:

PFUC:

VPS:

NVI:

TPRE:

MDL:

FAS:

CCIN:

DSM:

NPT:

NFB:

NLO:

NVG:

AROA:

TCA:

DPA:

AGRG:

ATD:

AGB:

LDC:

NVL:	108558092 108566586

APLN:

OTU:

BMOR:

BMAN:

MSEX:

DPL:	KGM_202065

BANY:

MJU:

NIQ:

VCD:

MCIX:

PMAC:

PRAP:

PBX:

PNAP:

ZCE:

CCRC:

LSIN:

AAGE:

HAW:

HZE:

TNL:

SLIU:

OFU:

PGW:

CFEL:

CCRN:

API:

DNX:

AGS:

RMD:

ACOO:

DVT:

BTAB:

CLEC:

HHAL:

HVI:

MQU:

BROR:

IEL:

DPX:	DAPPUDRAFT_230548

PCLA:

CQD:

MNZ:

HAZT:

EAF:

LSM:

TCF:

PPOI:

CEL:	CELE_Y53F4B.14(Y53F4B.14)

CBR:

CBG_20908

CRQ:	GCK72_006649

NAI:	NECAME_10277

PCAN:

BGT:

GAE:

OSN:

NVE:

EPA:

ATEN:

AMIL:

PDAM:

SPIS:

DGT:

XEN:

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