ATP6 polyclonal antibody

ATP6, also known as MT-ATP6, ATPASE6 or MTATP6, is a 226 amino acidmulti-passmembrane protein that localizestomitochondria and functions as a component of the F0 proton channel, playing a direct role in the transport of protons acrossthe mitochondrial membrane. Defectsin the gene encoding ATP6 are the cause of infantile bilateralstriatal necrosis, Leigh syndrome (LS),Leber hereditary optic neuropathy(LHON) and neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP), all of which are neurological or retinal disorders. The gene encoding ATP6 exists as part of themitochondrial genome.