Aliases for COX10 Gene
- Cytochrome C Oxidase Assembly Factor Heme A:Farnesyltransferase COX10 2 3 5
- Protoheme IX Farnesyltransferase, Mitochondrial 2 3 4
- Heme O Synthase 2 3 4
- COX10, Heme A:Farnesyltransferase Cytochrome C Oxidase Assembly Factor 2 3
- COX10 Homolog, Cytochrome C Oxidase Assembly Protein, Heme A: Farnesyltransferase (Yeast) 2
- COX10 Homolog, Cytochrome C Oxidase Assembly Protein, Heme A: Farnesyltransferase 3
- Cytochrome C Oxidase Assembly Homolog 10 (Yeast) 2
External Ids for COX10 Gene
Previous GeneCards Identifiers for COX10 Gene
Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion. [provided by RefSeq, Jul 2008]
GeneCards Summary for COX10 Gene
COX10 (Cytochrome C Oxidase Assembly Factor Heme A:Farnesyltransferase COX10) is a Protein Coding gene. Diseases associated with COX10 include Mitochondrial Complex Iv Deficiency, Nuclear Type 1 and Mitochondrial Complex Iii Deficiency, Nuclear Type 4. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and AMPK Enzyme Complex Pathway. Gene Ontology (GO) annotations related to this gene include cytochrome-c oxidase activity and prenyltransferase activity. An important paralog of this gene is COQ2.
UniProtKB/Swiss-Prot Summary for COX10 Gene
Converts protoheme IX and farnesyl diphosphate to heme O.