UQCRH

Chr 1p33

ubiquinol-cytochrome c reductase hinge protein

Aliases:: QCR6, UQCR8
MANE:: ENST00000311672.10

OMIM: 613844 ↗NCBI: 7388 ↗Ensembl ↗

Annotations refreshed 9 hours ago.

Predicted protein structure

AlphaFold predicted structure

UQCRH · P07919

v62025-08-01

Predicted Aligned Error matrix for UQCRH — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

87.6/ 100

Confident

91 residues

Confidence breakdown

Very high(≥ 90)68%
Confident(70–90)17%
Low(50–70)14%
Very low(< 50)1%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Moderate Evidence (Amber)

Mitochondrial disorder with complex III deficiency
BIALLELIC, autosomal or pseudoautosomal
Possible mitochondrial disorder - nuclear genes
Unknown
Likely inborn error of metabolism
Unknown
Mitochondrial disorders

Disease associations (Open Targets)

mitochondrial complex III deficiency, nuclear type 11
0.36
nonpapillary renal cell carcinoma
0.05
congenital bilateral absence of vas deferens
0.04
Hirschsprung disease
0.03
hepatocellular carcinoma
0.03
neoplasm
0.02
venous thromboembolism
0.02
retinitis pigmentosa 1
0.01
Alzheimer disease
0.01
cancer
0.01

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Cytochrome b-c1 complex subunit 6, mitochondrial

Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c