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Details on Person UniProt:P00846 MT-ATP6
| Class:Id | ReferenceGeneProduct:50499 |
|---|---|
| _chainChangeLog | chain:1-226 added on Fri February 6 2015 |
| _displayName | UniProt:P00846 MT-ATP6 |
| _timestamp | 2026-02-20 21:56:56 |
| chain | chain:1-226 |
| checksum | 7DB0F0BE86F55207 |
| comment | FUNCTION Subunit a, of the mitochondrial membrane ATP synthase complex (F(1)F(0) ATP synthase or Complex V) that produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain (Probable). ATP synthase complex consist of a soluble F(1) head domain - the catalytic core - and a membrane F(1) domain - the membrane proton channel (PubMed:37244256). These two domains are linked by a central stalk rotating inside the F(1) region and a stationary peripheral stalk (PubMed:37244256). During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation (Probable). With the subunit c (ATP5MC1), forms the proton-conducting channel in the F(0) domain, that contains two crucial half-channels (inlet and outlet) that facilitate proton movement from the mitochondrial intermembrane space (IMS) into the matrix (PubMed:37244256). Protons are taken up via the inlet half-channel and released through the outlet half-channel, following a Grotthuss mechanism (PubMed:37244256).CATALYTIC ACTIVITY H(+)(in) = H(+)(out)SUBUNIT Component of the ATP synthase complex composed at least of ATP5F1A/subunit alpha, ATP5F1B/subunit beta, ATP5MC1/subunit c (homooctamer), MT-ATP6/subunit a, MT-ATP8/subunit 8, ATP5ME/subunit e, ATP5MF/subunit f, ATP5MG/subunit g, ATP5MK/subunit k, ATP5MJ/subunit j, ATP5F1C/subunit gamma, ATP5F1D/subunit delta, ATP5F1E/subunit epsilon, ATP5PF/subunit F6, ATP5PB/subunit b, ATP5PD/subunit d, ATP5PO/subunit OSCP (PubMed:37244256). ATP synthase complex consists of a soluble F(1) head domain (subunits alpha(3) and beta(3)) - the catalytic core - and a membrane F(0) domain - the membrane proton channel (subunits c, a, 8, e, f, g, k and j) (PubMed:37244256). These two domains are linked by a central stalk (subunits gamma, delta, and epsilon) rotating inside the F1 region and a stationary peripheral stalk (subunits F6, b, d, and OSCP) (PubMed:37244256). Interacts with DNAJC30; interaction is direct (PubMed:30318146).SUBCELLULAR LOCATION The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the ATPase A chain family. |
| description | recommendedName: fullName evidence="33"ATP synthase F(0) complex subunit a alternativeName: F-ATPase protein 6 alternativeName: fullName evidence="33"Proton-conducting channel, ATP synthase F(0) complex subunit a |
| geneName | MT-ATP6 ATP6 ATPASE6 MTATP6 |
| identifier | P00846 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure ATP synthesis Cardiomyopathy CF(0) Disease variant Hydrogen ion transport Ion transport Leber hereditary optic neuropathy Leigh syndrome Membrane Mitochondrion Mitochondrion inner membrane Neuropathy Primary mitochondrial disease Proteomics identification Reference proteome Retinitis pigmentosa Transmembrane Transmembrane helix Transport |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | MT-ATP6 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8998179] ENSEMBL:ENSG00000198899 MT-ATP6 [Homo sapiens] |
| secondaryIdentifier | ATP6_HUMAN Q34772 Q5S8W5 Q5S9E7 Q5S9I6 Q5SA31 Q6RPB7 Q6VHC0 Q6VHE0 Q6WQF4 Q7YCC1 Q7YCF8 Q7YCG1 Q85KU8 Q85KX1 Q85L05 Q8HNQ4 Q8HNQ8 Q8WCX6 Q9B2U5 Q9B2Z2 |
| sequenceLength | 226 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:50498] MT-ATP6 [mitochondrial inner membrane] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:P00846 MT-ATP6 (50499)
