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Mitochondrial ATP Synthase (ATP synthase)
- Target
- ATP synthase
- Molecular classification
- Enzyme, ATP Synthase, Oxidative Phosphorylation Complex
01
Overview
Mitochondrial ATP synthase, also known as Complex V, is a large multi-subunit enzyme complex located in the inner mitochondrial membrane. It synthesizes ATP from ADP and inorganic phosphate, utilizing the energy stored in a transmembrane proton gradient. The enzyme consists of two main domains: F1 (catalytic) and F0 (proton channel). Mutations and oxidative stress can impair its function, leading to various diseases.
- Other names
- Complex VF1F0-ATPaseATP5A1ATP5BATP5C1ATP5DATP5E
02
Mechanism of action
Rotary catalysis driven by proton gradient, conformational changes in beta subunits facilitate ATP synthesis.
03
Biological functions
ATP synthesisEnergy productionProton transportOxidative phosphorylation
04
Disease associations
Neuromuscular disordersMitochondrial diseaseNeurodegenerative diseaseAlzheimer's disease
05
Safety considerations
Mutations in subunits can cause complex V deficiency.Oxidative stress can impair activity.Dysfunctional assembly can lead to disease phenotypes.
06
Interacting drugs
IF_1 (inhibitor protein)
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