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)

PTR:	740718(UQCC3)

PPS:

100993556

GGO:

101124279

PON:

100455801

MCC:	718716(UQCC3)

MCF:

102137003

MTHB:

126935534

MNI:

105469453

CSAB:

103233987(C1H11orf83)

CATY:

PANU:

TGE:

MLEU:

RRO:

RBB:

TFN:

PTEH:

CANG:

SBQ:

CIMI:

CSYR:

MMUR:

LCAT:

PCOQ:

OGA:

MMU:	107197(Uqcc3)

MCAL:

110285840

MPAH:

110334341

RNO:	120093241 690344(Uqcc3)

MCOC:

ANU:

MUN:

CGE:

MAUA:

PROB:

PLEU:

MORG:

AAMP:

NGI:

HGL:

CPOC:

CCAN:

DORD:

DSP:	122105127 122112995

NCAR:

MMMA:

OCU:

OPI:

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:

PTG:

PPAD:

PUC:

AJU:

HHV:

BTA:	515452(UQCC3)

BOM:

BIU:

BBUB:

BBIS:

CHX:

OAS:

BTAX:

ODA:

CCAD:

MBEZ:

SSC:

CFR:

CBAI:

CDK:

VPC:

BACU:

BMUS:

LVE:

OOR:

DLE:

PCAD:

PSIU:

NASI:

ECB:	100063691(UQCC3)

EPZ:

EAI:

MYB:

MYD:

MMYO:

MLF:

PKL:

EFUS:

MNA:

DRO:

SHON:

AJM:

PDIC:

PHAS:

MMF:

PPAM:

HAI:

RFQ:

PALE:

PGIG:

PVP:

MJV:

TOD:

SARA:

SETR:

LAV:

TMU:

ETF:

DNM:

MDO:

GAS:

SHR:

AFZ:

PCW:

OAA:

NMEL:

PMOA:

120496324 120511630(DMAC1)

OTC:	121344158 121344160

PRUF:

121364677(DMAC1)

OMA:

PHI:

PMAJ:

SVG:

HRT:

DNE:

PSS:

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

BBUF:

120993910

BGAR:

122932040

DRE:	558925(uqcc3)

SRX:	107718796 107720609

SANH:

107693944 107701168

SGH:	107548866 107555155

CCAR:

CAUA:

CGIB:

PTET:

LROH:

OMC:

PPRM:

RKG:

MAMB:

CIDE:

MASI:

TROS:

TDW:

MANU:

IPU:

IFU:

SMEO:

TFD:

TVC:

AMEX:

CMAO:

EEE:

CHAR:

TRU:

TNG:	GSTEN00034871G001

LCO:

TBEN:

PGEO:

GACU:

EMAC:

ELY:

EFO:

PLEP:

SLUC:

ECRA:

ESP:

PFLV:

GAT:

PPUG:

AFB:

CLUM:

PSWI:

SCHU:

ALAT:

MZE:

ONL:

OAU:

OLA:

OML:

CSAI:

XMA:

XCO:

XHE:

PRET:

PFOR:

PLAI:

PMEI:

GAF:

PPRL:

CTUL:

GMU:

KMR:

AOCE:

CSEM:

POV:

SSEN:

HHIP:

HSP:

SMAU:

LCF:

SDU:

SLAL:

XGL:

HCQ:

SSCV:

PEE:

BPEC:

BSPL:

SJO:

SASA:

100286673(yk001)

STRU:

OTW:

OMY:

OGO:

ONE:	115101340 115114847

OKI:

OKE:

SALP:

SNH:

CCLU:

ELS:

SFM:

PKI:

AANG:

LOC:

PSPA:

LCM:

CMK:

RTP:

HOC:

BBEL:

SPU:

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