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Mitochondrial Oxidative Phosphorylation Complex I (Complex I)
- Target
- Complex I
- Molecular classification
- Enzyme, Oxidoreductase, Mitochondrial respiratory chain complex
01
Overview
Mitochondrial oxidative phosphorylation complex I is the first and largest enzyme complex of the mitochondrial electron transport chain, located in the inner mitochondrial membrane. It catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q10), coupled to proton translocation across the membrane, contributing to ATP synthesis. Deficiency or dysfunction can cause severe multisystem disorders, and altered activity has been implicated in cancer cell survival.
- Other names
- NADH:ubiquinone oxidoreductaseNADH dehydrogenase
02
Mechanism of action
Inhibition of electron transfer from NADH to ubiquinone and/or proton translocation.
03
Biological functions
Electron transportProton translocationATP synthesisOxidative phosphorylationRedox control
04
Disease associations
Mitochondrial complex I deficiencyLeigh syndromeLeber hereditary optic neuropathyCancerNeurological disordersMuscular disordersCardiac disorders
05
Safety considerations
Increased reactive oxygen species productionApoptosisMultisystem disordersLactic acidosisNeurotoxicity
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