LMPD Database

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LMP000342

UniProt Annotations

Entry Information
Gene NameATP synthase, H+ transporting, mitochondrial F0 complex, subunit C2 (subunit 9)
Protein EntryAT5G2_MOUSE
UniProt IDP56383
SpeciesMouse
Comments
Comment typeDescription
DiseaseNote=This protein is the major protein stored in the storage bodies of animals or humans affected with ceroid lipofuscinosis (Batten disease).
FunctionMitochondrial 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. Part of the complex F(0) domain. A homomeric c-ring of probably 10 subunits is part of the complex rotary element.
MiscellaneousThere are three genes which encode the mitochondrial ATP synthase proteolipid and they specify precursors with different import sequences but identical mature proteins. {ECO:0000305}.
SimilarityBelongs to the ATPase C chain family. {ECO:0000305}.
Subcellular LocationMitochondrion membrane; Multi-pass membrane protein.
SubunitF-type ATPases have 2 components, CF(1) - the catalytic core - and CF(0) - the membrane proton channel. CF(1) has five subunits: alpha(3), beta(3), gamma(1), delta(1), epsilon(1). CF(0) has three main subunits: a, b and c.