MitoProteome Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins

MT000162

UniProt Annotations

Entry Information

Gene Name	cytochrome c-1
Protein Entry	CY1_HUMAN
UniProt ID	P08574
Species	Human

Comments

Comment type	Description
Disease	Mitochondrial complex III deficiency, nuclear 6 (MC3DN6) [MIM:615453]: An autosomal recessive disorder caused by mitochondrial dysfunction. It is characterized by onset in early childhood of episodic acute lactic acidosis, ketoacidosis, and insulin-responsive hyperglycemia, usually associated with infection. Laboratory studies show decreased activity of mitochondrial complex III. Psychomotor development is normal. {ECO:0000269\|PubMed:23910460}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Function	This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain.
Ptm	Binds 1 heme group per subunit.
Similarity	Belongs to the cytochrome c family. {ECO:0000305}.
Similarity	Contains 1 cytochrome c domain. {ECO:0000305}.
Subcellular Location	Mitochondrion inner membrane; Single-pass membrane protein; Intermembrane side.
Subunit	The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).
Web Resource	Name=NIEHS-SNPs; URL="http://egp.gs.washington.edu/data/cyc1/";