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"ATP6" redirects here. For the nuclear genes, see V-ATPase and ATPase.
ATP synthase A chain
Symbol ATP-synt_A
Pfam PF00119
InterPro IPR000568
SCOP 1c17
OPM superfamily 5
OPM protein 1c17
ATP synthase F0 subunit 6
Symbols ATP6 ; ATPase6; MTATP6
External IDs OMIM516060 MGI99927 HomoloGene5012 IUPHAR: GeneCards: ATP6 Gene
EC number
Species Human Mouse
Entrez 4508 17705
Ensembl ENSG00000198899 ENSMUSG00000064357
UniProt P00846 P00848
RefSeq (mRNA) n/a n/a
RefSeq (protein) n/a NP_904333
Location (UCSC) Chr MT:
0.01 – 0.01 Mb
Chr MT:
0.01 – 0.01 Mb
PubMed search [1] [2]

ATP synthase F0 subunit 6 (or subunit/chain A) (human mitochondrial gene name ATP6) is a subunit of F0 complex of transmembrane F-type ATP synthase.[1]


This subunit is a key component of the proton channel, and may play a direct role in the translocation of protons across the membrane. Catalysis in the F1 complex depends upon the rotation of the central stalk and F0 c-ring, which in turn is driven by the flux of protons through the membrane via the interface between the F0 c-ring and subunit A. The peripheral stalk links subunit A to the external surface of the F1 domain, and is thought to act as a stator to counter the tendency of subunit A and the F1alpha(3)beta(3) catalytic portion to rotate with the central rotary element.[2]

3D structure of E. coli homologue of this subunit was modelled based on electron microscopy data (chain M of PDB: 1c17 ). It forms a transmembrane 4-α-bundle.

Clinical significance[edit]

ATP6 is a gene associated with neuropathy, ataxia, and retinitis pigmentosa.[3]


  1. ^ Anderson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG (April 1981). "Sequence and organization of the human mitochondrial genome". Nature 290 (5806): 457–65. doi:10.1038/290457a0. PMID 7219534. 
  2. ^ Walker JE, Runswick MJ, Neuhaus D, Montgomery MG, Carbajo RJ, Kellas FA (2005). "Structure of the F1-binding domain of the stator of bovine F1Fo-ATPase and how it binds an alpha-subunit". J. Mol. Biol. 351 (4): 824–838. doi:10.1016/j.jmb.2005.06.012. PMID 16045926. 
  3. ^ Baracca A, Sgarbi G, Mattiazzi M, Casalena G, Pagnotta E, Valentino ML, Moggio M, Lenaz G, Carelli V, Solaini G (July 2007). "Biochemical phenotypes associated with the mitochondrial ATP6 gene mutations at nt8993". Biochim. Biophys. Acta 1767 (7): 913–9. doi:10.1016/j.bbabio.2007.05.005. PMID 17568559. 

Further reading[edit]

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