Target intelligence / Profile preview

Mitochondrial Respiratory Complex I

Molecular classification
Enzyme, Protein Complex
01

Overview

Mitochondrial Respiratory Complex I, also known as NADH:ubiquinone oxidoreductase, is the first and largest enzyme of the mitochondrial electron transport chain. It catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q), coupled with proton translocation across the inner mitochondrial membrane. Dysfunction is linked to various hereditary and degenerative diseases due to impaired oxidative phosphorylation or increased reactive oxygen species (ROS) generation.

Other names
Protein complex 1NADH:ubiquinone oxidoreductaseComplex I
02

Mechanism of action

Inhibition of electron transfer, disruption of proton gradient

03

Biological functions

Electron transportProton transportOxidative phosphorylationEnergy production
04

Disease associations

Neurodegenerative diseaseHereditary disordersMitochondrial dysfunction
05

Safety considerations

Increased ROS generationImpaired oxidative phosphorylation

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