Uqcrfs1 Gene Summary [Mouse]

Predicted to enable oxidoreductase activity. Predicted to be involved in mitochondrial electron transport, ubiquinol to cytochrome c. Located in mitochondrion. Part of respiratory chain complex III. Is active in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; cardiovascular system; hemolymphoid system; and liver. Used to study mitochondrial metabolism disease. Human ortholog(s) of this gene implicated in mitochondrial complex III deficiency. Orthologous to several human genes including UQCRFS1 (ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1). [provided by Alliance of Genome Resources, Jul 2025]

Details

Type
Protein Coding
Official Symbol
Uqcrfs1
Official Name
ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [Source:MGI Symbol;Acc:MGI:1913944]
Ensembl ID
ENSMUSG00000038462
Bio databases IDs NCBI: 66694 Ensembl: ENSMUSG00000038462
Aliases ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
Synonyms 4430402G14RIK, Complex III FeS, Fes subunit of complex iii, ISP, LRRGT00195, MC3DN10, Rieske, RIP1, RIS1, RISP, UBIQUINOL CYTOCHROME C REDUCTASE, ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1, ubiquinol-cytochrome c reductase, Rieske iron-sulphur polypeptide 1, UCRI, UQCR5
Species
Mouse, Mus musculus
OrthologiesHumanRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Uqcrfs1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • Rieske
  • ubiquinol-cytochrome-c reductase
  • binding protein
  • Ubiquinol cytochrome reductase transmembrane region
  • enzyme
  • protein binding
  • ubiquinol-cytochrome c reductase, iron-sulfur subunit
  • Ubiquinol-cytochrome c reductase 8 kDa, N-terminal

Pathways

Biological processes and signaling networks where the Uqcrfs1 gene in mouse plays a role, providing insight into its function and relevance in health or disease.

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • Parkinson disease
  • Huntington disease
  • organismal death
  • ovarian cancer
  • nuclear type 10 mitochondrial complex III deficienc
  • progressive familial intrahepatic cholestasis type 1
  • psoriasis
  • infection by HIV-1
regulated by
regulates
role in cell
  • expression in
  • proliferation
  • apoptosis
  • production in
  • activation in
  • cell death
  • phosphorylation in
  • number
  • quantity
  • flux in

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • myelin enriched fraction
  • pH resistant lipid raft fraction
  • Mitochondria
  • cytosol
  • mitochondrial membrane
  • mitochondrial matrix
  • mitochondrial inner membrane

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the mouse Uqcrfs1 gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • mitochondrial respiratory chain complex III assembly
  • respiratory electron transport chain
  • mitochondrial electron transport, ubiquinol to cytochrome c

Cellular Component

Where in the cell the gene product is active
  • mitochondrion
  • mitochondrial respiratory chain complex III
  • mitochondrial respiratory chain complex IV
  • mitochondrial inner membrane

Molecular Function

What the gene product does at the molecular level
  • protein binding
  • metal ion binding
  • oxidoreductase activity
  • ubiquinol-cytochrome-c reductase activity
  • 2 iron, 2 sulfur cluster binding

Gene-Specific Assays for Results You Can Trust

Streamline your workflow with assays designed for this gene. Our targeted dPCR and qPCR assays help you generate meaningful data – efficiently and accurately.