Entity Details

Primary name UQCC3_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ6UW78
EntryNameUQCC3_HUMAN
FullNameUbiquinol-cytochrome-c reductase complex assembly factor 3
TaxID9606
Evidenceevidence at protein level
Length93
SequenceStatuscomplete
DateCreated2004-11-23
DateModified2021-06-02

Ontological Relatives

GenesUQCC3

GO terms

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GOName
GO:0005654 nucleoplasm
GO:0005739 mitochondrion
GO:0005829 cytosol
GO:0006122 mitochondrial electron transport, ubiquinol to cytochrome c
GO:0006754 ATP biosynthetic process
GO:0031305 integral component of mitochondrial inner membrane
GO:0034551 mitochondrial respiratory chain complex III assembly
GO:0042407 cristae formation
GO:0070300 phosphatidic acid binding
GO:1901612 cardiolipin binding

Subcellular Location

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Subcellular Location
Mitochondrion inner membrane

Domains

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DomainNameCategoryType
IPR027896 Ubiquinol-cytochrome-c reductase complex assembly factor 3FamilyFamily

Diseases

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Disease IDSourceNameDescription
616111 OMIMMitochondrial complex III deficiency, nuclear 9 (MC3DN9)A form of mitochondrial complex III deficiency, a disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. MC3DN9 clinical features include feeding difficulties, hypoglycemia, severe lactic acidosis, and delayed psychomotor development. The disease is caused by variants affecting the gene represented in this entry.

Interactions

1 interaction

InteractorPartnerSourcesPublicationsLink
UQCC3_HUMANPHLA3_HUMANIntAct17353931 details