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Details on Person UniProt:P00846 MT-ATP6

Class:IdReferenceGeneProduct:50499
_chainChangeLogchain:1-226 added on Fri February 6 2015
_displayNameUniProt:P00846 MT-ATP6
_timestamp2026-02-20 21:56:56
chainchain:1-226
checksum7DB0F0BE86F55207
commentFUNCTION 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.
descriptionrecommendedName: 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
geneNameMT-ATP6
ATP6
ATPASE6
MTATP6
identifierP00846
isSequenceChangedFALSE
keyword3D-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
nameMT-ATP6
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998179] ENSEMBL:ENSG00000198899 MT-ATP6 [Homo sapiens]
secondaryIdentifierATP6_HUMAN
Q34772
Q5S8W5
Q5S9E7
Q5S9I6
Q5SA31
Q6RPB7
Q6VHC0
Q6VHE0
Q6WQF4
Q7YCC1
Q7YCF8
Q7YCG1
Q85KU8
Q85KX1
Q85L05
Q8HNQ4
Q8HNQ8
Q8WCX6
Q9B2U5
Q9B2Z2
sequenceLength226
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:50498] MT-ATP6 [mitochondrial inner membrane] [Homo sapiens]
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