UQCC3

Protein identityUniProt · HPA

UniProt accession

Q6UW78

Gene

UQCC3

Ensembl

ENSG00000204922

Chromosome

11

Canonical length

93 aa

Protein class

Disease related genes, Human disease related genes, Predicted membrane proteins

Subcellular location

Nucleoplasm,Mitochondria,Cytosol

OverviewNCBI Gene

Complex III is a mitochondrial inner membrane protein complex that transfers electrons from ubiquinol to cytochrome c. This gene encodes a protein that functions in complex III assembly. Mutations in this gene result in Mitochondrial complex III deficiency, nuclear type 9. [provided by RefSeq, Dec 2014]

Canonical amino-acid sequenceUniProt

93 residues, UniProt reviewed canonical sequence.

>Q6UW78|UQCC3
     1  MDSLRKMLIS VAMLGAGAGV GYALLVIVTP GERRKQEMLK EMPLQDPRSR EEAARTQQLL
    61  LATLQEAATT QENVAWRKNW MVGGEGGAGG RSP

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against UQCC3 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Other membrane

Secreted

No

Transmembrane segments

1

Mean surface accessibility (rSASA)

0.57

Highest tissue expression

15 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about UQCC3.

Disease | AllUniProt

Conditions UQCC3 is implicated in, by any mechanism.

• Mitochondrial complex III deficiency, nuclear type 9 (MC3DN9) MIM:616111

Disease | GeneticClinVar

1 pathogenic / likely-pathogenic of 51 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial complex III deficiency nuclear type 9

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

1.83

gnomAD pLI

0.03

DepMap mean gene effect

-0.36

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• ATP biosynthetic process
• cristae formation
• mitochondrial electron transport, ubiquinol to cytochrome c
• mitochondrial respiratory chain complex III assembly

Molecular functions

• cardiolipin binding
• phosphatidic acid binding

Cellular components

• cytosol
• mitochondrial inner membrane
• mitochondrion
• nucleoplasm

Protein domainsUniProt · Pfam · InterPro

• Ubiquinol-cytochrome-c reductase complex assembly factor 3
• Ubiquinol-cytochrome-c reductase complex assembly factor 3

KeywordsUniProt

• ATP synthesis
• Membrane
• Mitochondrion
• Mitochondrion inner membrane
• Primary mitochondrial disease
• Transmembrane
• Transmembrane helix

InteractionsUniProt · HPA

Protein binding partners of UQCC3 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• UQCC1