ATP synthase subunit a

Details

Name
ATP synthase subunit a
Synonyms
  • ATP6
  • ATPASE6
  • F-ATPase protein 6
  • MTATP6
Gene Name
MT-ATP6
Organism
Humans
Amino acid sequence
>lcl|BSEQ0049741|ATP synthase subunit a
MNENLFASFIAPTILGLPAAVLIILFPPLLIPTSKYLINNRLITTQQWLIKLTSKQMMTM
HNTKGRTWSLMLVSLIIFIATTNLLGLLPHSFTPTTQLSMNLAMAIPLWAGTVIMGFRSK
IKNALAHFLPQGTPTPLIPMLVIIETISLLIQPMALAVRLTANITAGHLLMHLIGSATLA
MSTINLPSTLIIFTILILLTILEIAVALIQAYVFTLLVSLYLHDNT
Number of residues
226
Molecular Weight
24816.865
Theoretical pI
Not Available
GO Classification
Functions
hydrogen ion transmembrane transporter activity / transmembrane transporter activity
Processes
aging / ATP biosynthetic process / mitochondrial ATP synthesis coupled proton transport / response to hyperoxia
Components
extracellular exosome / integral component of membrane / mitochondrial inner membrane / mitochondrial proton-transporting ATP synthase complex / proton-transporting ATP synthase complex, coupling factor F(o)
General Function
Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) 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. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. 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. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane.
Specific Function
Hydrogen ion transmembrane transporter activity
Pfam Domain Function
Transmembrane Regions
6-26 68-88 97-117 138-158 164-184 189-209
Cellular Location
Mitochondrion inner membrane
Gene sequence
>lcl|BSEQ0049742|ATP synthase subunit a (MT-ATP6)
ATGAACGAAAATCTGTTCGCTTCATTCATTGCCCCCACAATCCTAGGCCTACCCGCCGCA
GTACTGATCATTCTATTTCCCCCTCTATTGATCCCCACCTCCAAATATCTCATCAACAAC
CGACTAATCACCACCCAACAATGACTAATCAAACTAACCTCAAAACAAATGATAACCATA
CACAACACTAAAGGACGAACCTGATCTCTTATACTAGTATCCTTAATCATTTTTATTGCC
ACAACTAACCTCCTCGGACTCCTGCCTCACTCATTTACACCAACCACCCAACTATCTATA
AACCTAGCCATGGCCATCCCCTTATGAGCGGGCACAGTGATTATAGGCTTTCGCTCTAAG
ATTAAAAATGCCCTAGCCCACTTCTTACCACAAGGCACACCTACACCCCTTATCCCCATA
CTAGTTATTATCGAAACCATCAGCCTACTCATTCAACCAATAGCCCTGGCCGTACGCCTA
ACCGCTAACATTACTGCAGGCCACCTACTCATGCACCTAATTGGAAGCGCCACCCTAGCA
ATATCAACCATTAACCTTCCCTCTACACTTATCATCTTCACAATTCTAATTCTACTGACT
ATCCTAGAAATCGCTGTCGCCTTAATCCAAGCCTACGTTTTCACACTTCTAGTAAGCCTC
TACCTGCACGACAACACATAA
Chromosome Location
Not Available
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDP00846
UniProtKB Entry NameATP6_HUMAN
HGNC IDHGNC:7414
General References
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  23. Lamminen T, Majander A, Juvonen V, Wikstrom M, Aula P, Nikoskelainen E, Savontous ML: A mitochondrial mutation at nt 9101 in the ATP synthase 6 gene associated with deficient oxidative phosphorylation in a family with Leber hereditary optic neuroretinopathy. Am J Hum Genet. 1995 May;56(5):1238-40. [Article]
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  25. Campos Y, Martin MA, Rubio JC, Solana LG, Garcia-Benayas C, Terradas JL, Arenas J: Leigh syndrome associated with the T9176C mutation in the ATPase 6 gene of mitochondrial DNA. Neurology. 1997 Aug;49(2):595-7. [Article]
  26. Takahashi S, Makita Y, Oki J, Miyamoto A, Yanagawa J, Naito E, Goto Y, Okuno A: De novo mtDNA nt 8993 (T-->G) mutation resulting in Leigh syndrome. Am J Hum Genet. 1998 Mar;62(3):717-9. [Article]
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  28. Rieder MJ, Taylor SL, Tobe VO, Nickerson DA: Automating the identification of DNA variations using quality-based fluorescence re-sequencing: analysis of the human mitochondrial genome. Nucleic Acids Res. 1998 Feb 15;26(4):967-73. [Article]
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  31. Ware SM, El-Hassan N, Kahler SG, Zhang Q, Ma YW, Miller E, Wong B, Spicer RL, Craigen WJ, Kozel BA, Grange DK, Wong LJ: Infantile cardiomyopathy caused by a mutation in the overlapping region of mitochondrial ATPase 6 and 8 genes. J Med Genet. 2009 May;46(5):308-14. doi: 10.1136/jmg.2008.063149. Epub 2009 Feb 2. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00783Estradiolapproved, investigational, vet_approvedunknowninhibitorDetails
DB13952Estradiol acetateapproved, investigational, vet_approvedunknownDetails
DB13953Estradiol benzoateapproved, investigational, vet_approvedunknownDetails
DB13954Estradiol cypionateapproved, investigational, vet_approvedunknownDetails
DB13955Estradiol dienanthateapproved, investigational, vet_approvedunknownDetails
DB13956Estradiol valerateapproved, investigational, vet_approvedunknownDetails