ATP5PO acetyl K139
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(0) domain and the peripheric stalk, which acts as a stator to hold the catalytic alpha(3)beta(3) subcomplex and subunit a/ATP6 static relative to the rotary elements.
Involvement in disease
Mitochondrial complex V deficiency, nuclear type 7
MC5DN7
An autosomal recessive, severe, mitochondrial disorder apparent soon after birth. It is characterized by multisystemic features that include hypotonia, developmental delay, progressive epileptic encephalopathy, progressive cerebral atrophy, white matter abnormalities on brain imaging, and hypertrophic cardiomyopathy in some patients. Death in infancy or early childhood may occur.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Acetylation at Lys-162 decreases ATP production. Deacetylated by SIRT3.
In response to mitochondrial stress, the precursor protein is ubiquitinated by the SIFI complex in the cytoplasm before mitochondrial import, leading to its degradation (PubMed:38297121). Within the SIFI complex, UBR4 initiates ubiquitin chain that are further elongated or branched by KCMF1 (PubMed:38297121).
Sequence Similarities
Belongs to the ATPase delta chain family.
Cellular localization
- Mitochondrion
- Mitochondrion inner membrane
Alternative names
ATP5O, ATPO, ATP5PO, ATP synthase peripheral stalk subunit OSCP, Oligomycin sensitivity conferral protein, OSCP