MT-ATP6

MT-ATP6

ATP synthase A chain
Identifiers
Symbol ATP-synt_A
Pfam InterPro PROSITE PDOC00420
SCOP SUPERFAMILY OPM superfamily OPM protein 1c17
ATP synthase F0 subunit 6
Identifiers
3.6.3.14
Orthologs
SpeciesHumanMouse

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]

Function

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 1c17). It forms a transmembrane 4-α-bundle.

Clinical significance

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

References


Further reading


External links

  • GeneReviews/NCBI/NIH/UW entry on Mitochondrial DNA-Associated Leigh Syndrome and NARP
  • Medical Subject Headings (MeSH)