{"metadata":{"accession":"PF12597","entry_id":null,"type":"family","go_terms":null,"source_database":"pfam","member_databases":null,"integrated":"IPR022533","hierarchy":null,"name":{"name":"Cytochrome c oxidase assembly protein COX20","short":"Cox20"},"description":[{"text":"<p>Complex IV (CIV) is a copper-heme oxidase that couples electron transfer from cytochrome c to oxygen with proton extrusion across the inner membrane to contribute to the proton gradient required for ATP generation. This entry includes fungal mitochondrial complex IV assembly factor Cox20 [[cite:PUB00053731]] and its homologues (also known as FAM36A in metazoa [[cite:PUB00086694]]. Human CIV is formed by three catalytic core subunits (COX1/2/3). COX20 has been found to form a complex with SCO1, SCO2, and newly synthesized COX2. It may act as a chaperone in the early steps of COX2 maturation [[cite:PUB00088297]].</p>","llm":false,"checked":false,"updated":false}],"wikipedia":null,"literature":{"PUB00053731":{"PMID":10671482,"ISBN":null,"volume":"275","issue":"7","year":2000,"title":"Identification of Cox20p, a novel protein involved in the maturation and assembly of cytochrome oxidase subunit 2.","URL":null,"raw_pages":"4571-8","medline_journal":"J Biol Chem","ISO_journal":"J. Biol. Chem.","authors":["Hell K","Tzagoloff A","Neupert W","Stuart RA."],"DOI_URL":"http://dx.doi.org/10.1074/jbc.275.7.4571"},"PUB00086694":{"PMID":23125284,"ISBN":null,"volume":"22","issue":"4","year":2013,"title":"A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia.","URL":null,"raw_pages":"656-67","medline_journal":"Hum Mol Genet","ISO_journal":"Hum. Mol. Genet.","authors":["Szklarczyk R","Wanschers BF","Nijtmans LG","Rodenburg RJ","Zschocke J","Dikow N","van den Brand MA","Hendriks-Franssen MG","Gilissen C","Veltman JA","Nooteboom M","Koopman WJ","Willems PH","Smeitink JA","Huynen MA","van den Heuvel LP."],"DOI_URL":"https://doi.org/10.1093/hmg/dds473"},"PUB00088297":{"PMID":24403053,"ISBN":null,"volume":"23","issue":"11","year":2014,"title":"Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.","URL":null,"raw_pages":"2901-13","medline_journal":"Hum Mol Genet","ISO_journal":"Hum. Mol. Genet.","authors":["Bourens M","Boulet A","Leary SC","Barrientos A."],"DOI_URL":"https://doi.org/10.1093/hmg/ddu003"}},"set_info":null,"overlaps_with":null,"counters":{"subfamilies":0,"domain_architectures":29,"interactions":0,"matches":2679,"pathways":0,"proteins":2661,"proteomes":2417,"sets":0,"structural_models":{"alphafold":2317},"structures":0,"taxa":6450},"entry_annotations":{"hmm":0,"logo":0,"alignment:seed":52,"alignment:full":2112},"cross_references":{},"is_llm":false,"is_reviewed_llm":false,"is_updated_llm":false,"representative_structure":null}}