Mitochondrial ATP synthases (ATPases) transduce the energy contained in membrane electrochemical proton gradients into the energy required for synthesis of high-energy phosphate bonds. ATPases contain two linked complexes: F1, the hydrophilic catalytic core; and F0, the membrane-embedded protein channel. F1 consists of three Alpha chains and three Beta chains, which are weakly homologous, as well as one Gamma chain, one Delta chain and one e chain. F0 consists of three subunits: a, b and c. The e chain of F1 contains 50 amino acids and is the smallest of the five ATPase F1 chains. Mitochondrial ATPase e chain (ATP5E) localizes to the mitochondria and catalyzes ATP synthesis.
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. Part of the complex F(1) domain and of the central stalk which is part of the complex rotary element. Rotation of the central stalk against the surrounding alpha(3)beta(3) subunits leads to hydrolysis of ATP in three separate catalytic sites on the beta subunits.
Subunit:
F-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) seems to have nine subunits: a, b, c, d, e, f, g, F6 and 8 (or A6L). Component of an ATP synthase complex composed of ATP5F1, ATP5G1, ATP5E, ATP5H, ATP5I, ATP5J, ATP5J2, MT-ATP6, MT-ATP8, ATP5A1, ATP5B, ATP5D, ATP5C1, ATP5O, ATP5L, USMG5 and MP68 (By similarity).
Subcellular Location:
Mitochondrion. Mitochondrion inner membrane.
Tissue Specificity:
Ubiquitous.
DISEASE:
Defects in ATP5E are the cause of mitochondrial complex V deficiency nuclear type 3 (MC5DN3) [MIM:614053]. MC5DN3 is a mitochondrial disorder with heterogeneous clinical manifestations including dysmorphic features, psychomotor retardation, hypotonia, growth retardation, cardiomyopathy, enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebrospinal fluid.
Similarity:
Belongs to the eukaryotic ATPase epsilon family.
SWISS:
P56381
Gene ID:
514
Database links:
SwissProt ：P56381
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