CYC1

Also known as MC3DN6, UQCR4, cytochrome c1

Cytochrome c1, heme protein, mitochondrial (CYC1), also known as UQCR4, MC3DN6, Complex III subunit 4, Cytochrome b-c1 complex subunit 4, or Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit is a protein that in humans is encoded by the CYC1 gene. CYC1 is a respiratory subunit of Ubiquinol Cytochrome c Reductase (complex III), which is located in the inner mitochondrial membrane and is part of the electron transport chain. Mutations in this gene may cause mitochondrial complex III deficiency, nuclear, type 6.

Gene data

CYC1

Name: cytochrome c1
Type: protein-coding
Position: 144,095,039–144,097,525 (+)
Aliases: MC3DN6, UQCR4
Ensembl: ENSG00000179091
RefSeq RNA: NM_001916.5
RefSeq protein: NP_001907.3

This gene encodes a subunit of the cytochrome bc1 complex, which plays an important role in the mitochondrial respiratory chain by transferring electrons from the Rieske iron-sulfur protein to cytochrome c. Mutations in this gene may cause mitochondrial complex III deficiency, nuclear type 6. [provided by RefSeq, Dec 2013].

Gene Ontology

Biological process

mitochondrial electron transport, ubiquinol to cytochrome c mitochondrial electron transport, ubiquinol to cytochrome c response to glucagon

Article

8 sections

Contents

Structure
Function
Species
Clinical Significance
Interactions
References
External links
Further reading

== Structure == CYC1 is located on the q arm of chromosome 8 in position 24.3 and has 8 exons. The CYC1 gene produces a 13.5 kDa protein composed of 130 amino acids. CYC1 belongs to the cytochrome c family. CYC1 is a phosphoprotein and subunit of Ubiquinol Cytochrome c Reductase that binds heme groups. It has helix, transit peptide, and transmembrane domains and contains 9 alpha helixes, 5 beta strands, and 3 turns. The transmembrane protein passes through the inner mitochondrial membrane once and the majority of the protein is found on the intermembrane side. CYC1 contains covalent heme bindings sites at positions 121 and 124 and heme axial ligand iron-metal binding sites at positions 125 and 244.