KEGG ORTHOLOGY: K23165

ORTHOLOGY: K23165

Entry

K23165                      KO

Symbol

UQCC3

Name

ubiquinol-cytochrome-c reductase complex assembly factor 3

Disease

H02086

Mitochondrial complex III deficiency

Brite

KEGG Orthology (KO) [BR:ko00001]
09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K23165  UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
Mitochondrial biogenesis [BR:ko03029]
Mitochondrial quality control factors
  Mitochondrial respiratory chain complex assembly factors
   Complex-III assembly factors
    K23165  UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3

Other DBs

GO:

1901612

Genes

HSA:	790955(UQCC3)

PPS:

GGO:

PON:

PPYG:

MCC:	718716(UQCC3)

MCF:	102137003(UQCC3)

MTHB:

126935534

MNI:

105469453

CSAB:

103233987(C1H11orf83)

CATY:

PANU:

TGE:

MLEU:

RRO:

RBB:

TFN:

PTEH:

CANG:

SBQ:

CIMI:

ANAN:

CSYR:

MMUR:

LCAT:

PCOQ:

OGA:

MMU:	107197(Uqcc3)

MCAL:

110285840

MPAH:

110334341

RNO:	120093241 690344(Uqcc3)

MCOC:

ANU:

ASYL:

MUN:

CGE:

MAUA:

PROB:

PLEU:

MORG:

AAMP:

NGI:

HGL:

CPOC:

CCAN:

DORD:

DSP:	122105127 122112995

DMER:

NCAR:

MMMA:

ITI:

OCU:

OPI:

OCZ:

TUP:

GVR:

CFA:	612210(UQCC3)

CLUD:

VVP:

VLG:

NPO:

AML:

UMR:

UAH:

UAR:

ELK:

LLV:

MPUF:

MNP:

MLK:

NVS:

ORO:

EJU:

ZCA:

MLX:

NSU:

LWW:

FCA:	101087173(UQCC3)

PYU:

PCOO:

PBG:

PVIV:

LRUF:

LGF:

AJU:

PTG:

PPAD:

PUC:

PLEZ:

HHV:

BTA:	515452(UQCC3)

BOM:	102271000(UQCC3)

BIU:

BBUB:

BBIS:

CHX:

OAS:

BTAX:

CSUM:

138084442(UQCC3)

ODA:

120874572

CCAD:

122430596

MREE:

136169636(UQCC3)

OVR:	110140874(UQCC3)

MBEZ:

SSC:

CFR:

CBAI:

CDK:

VPC:

BACU:

BMUS:

LVE:

OOR:

LALB:

GMF:	115840642(UQCC3)

DDP:	132429991(UQCC3)

DLE:

111183858

PCAD:

102973918

PSIU:

116757340

PPHC:

136127585(UQCC3)

NASI:

112402209

ECB:	100063691(UQCC3)

EPZ:	103558938(UQCC3)

EAI:

MYB:

MYD:

MMYO:

MLF:

MDT:

MYUM:

139018651(UQCC3)

PKL:

EFUS:

MNA:

DRO:

SHON:

AJM:

PDIC:

PHAS:

MMF:

PPAM:

HAI:

RFQ:

PALE:

PGIG:

PVP:

RAY:

MJV:

TOD:

SARA:

SFUM:

SETR:

LAV:

TMU:

ETF:

DNM:

MDO:

GAS:

SHR:

AFZ:

PCW:

TVP:

PBRV:

138144747(UQCC3)

OAA:

114810153

TACU:

119943811

NMEL:

110392031

PMOA:

120496324 120511630(DMAC1)

OTC:	121344158 121344160

PRUF:

121364677(DMAC1)

ATRC:

139587112(UQCC3)

OMA:

130262914

PHI:

106628976

PMAJ:

107199402

ACOE:

138100356(UQCC3)

ZLE:	135460268(DMAC1)

SVG:

106863540

HRT:

120748375

CCIC:

134055660

CBOY:

140660176(UQCC3)

CNIC:

CUCA:

CRID:

DNE:

PSS:

CIZ:	142019082(UQCC3)

CMY:

DCC:

CPIC:

TST:

CABI:

MRV:

ACS:

ASAO:

SUND:

121915912(DMAC1)

VKO:

PMUA:

PRAF:

ZVI:

HCG:

GJA:

STOW:

EMC:

XLA:	734185(uqcc3.L)

XTR:	106557438(uqcc3)

NPR:

108801958

ACAT:

141127489(UQCC3)

BBUF:

120993910

BGAR:

122932040

DTC:	141076152(UQCC3)

PWL:	138259441(UQCC3)

DRE:	558925(uqcc3)

SRX:	107718796 107720609

SANH:

107693944 107701168

SGH:	107548866 107555155

CCAR:

CAUA:

CGIB:

PTET:

LROH:

GRF:	141299239(uqcc3)

OMC:

PPRM:

RKG:

MAMB:

CIDE:

CERY:

137029654(uqcc3)

MASI:

127432531 127433749

TROS:

130545989

TDW:

130411993

MANU:

129446910(uqcc3)

PDAB:

135738350(uqcc3)

IPU:

IFU:

SMEO:

TFD:

TVC:

CGAR:

NGRA:

TRN:

AMEX:

CMAO:

EEE:

CHAR:

SPIL:

ASAD:

TRU:

TNG:	GSTEN00034871G001

LCO:

104926066

TBEN:

117473094

PGEO:

117452756(uqcc3)

GACU:

EMAC:

ELY:

EFO:

PLEP:

SLUC:

ECRA:

ESP:

PFLV:

GAT:

PPUG:

AFB:

CLUM:

PSWI:

CANA:

137778960(uqcc3)

CVE:	137890166(uqcc3)

SCHU:

122875946

SPUL:

138610729(uqcc3)

LMIX:

132973852

HTC:	141790707(uqcc3)

ALAT:

119019447

ASTR:

137595529(uqcc3)

MZE:

ONL:

OAU:

OLA:

OML:

CSAI:

XMA:

XCO:

XHE:

PRET:

PFOR:

PLAI:

PMEI:

GAF:

PPRL:

CTUL:

GMU:

KMR:

AOCE:

EJA:	138195710(uqcc3)

BFRE:

138636801(uqcc3)

CSEM:

POV:

SSEN:

SSOE:

HHIP:

HSP:

PPLT:

SMAU:

LCF:

SDU:

SLAL:

XGL:

HCQ:

SSCV:

125965757(uqcc3)

PEE:

133400463

BPEC:

110172437

ENY:	140346601(uqcc3)

BSPL:

114845400

SJO:

128365326

SSCO:

133979530

TTY:	137190505(uqcc3)

GMH:

115553774

SASA:

100286673(yk001)

STRU:

OTW:

OMY:

OGO:

ONE:	115101340 115114847

OKI:

OKE:

OMM:

OCLA:

SALP:

SNH:

SFON:

CCLU:

ELS:

OEP:

SFM:

PKI:

AANG:

CCOG:

LOC:

PSPA:

LCM:

CMK:

RTP:

HOC:

SCAU:

HAKA:

140717473(uqcc3)

BBEL:

SPU:

LVG:

PFLL:

DPO:

DPE:

DMN:

DHE:

HSY:

Reference

PMID:25008109

Authors

Wanschers BF, Szklarczyk R, van den Brand MA, Jonckheere A, Suijskens J, Smeets R, Rodenburg RJ, Stephan K, Helland IB, Elkamil A, Rootwelt T, Ott M, van den Heuvel L, Nijtmans LG, Huynen MA

Title

A mutation in the human CBP4 ortholog UQCC3 impairs complex III assembly, activity and cytochrome b stability.

Journal

Hum Mol Genet 23:6356-65 (2014)
DOI:10.1093/hmg/ddu357

Sequence

[hsa:790955]

LinkDB

DBGET integrated database retrieval system