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NADH-ubiquinone oxidoreductase chain I

Molecular classification
Enzyme, Electron transport chain enzyme
01

Overview

NADH-ubiquinone oxidoreductase (complex I) is the first and largest enzyme complex of the mitochondrial respiratory chain. It is embedded in the inner mitochondrial membrane and consists of at least 45 subunits in mammals, encoded by both nuclear and mitochondrial DNA. Its core function is to transfer electrons from NADH to ubiquinone (coenzyme Q), coupled with the translocation of protons across the inner mitochondrial membrane, helping generate the proton gradient used for ATP production through oxidative phosphorylation. Defects or inhibition of complex I can lead to mitochondrial diseases, contribute to neurodegenerative and cardiovascular diseases, and increase cellular oxidative stress[1][3][5][4][7]. Note on correctness ("is_incorrect"): "NADH dehydrogenase chain I" by itself is not a standard canonical target name; it is ambiguous, as it can refer to: - The ND1 subunit (NADH-ubiquinone oxidoreductase chain 1, often abbreviated as ND1), which is one mitochondrial DNA-encoded subunit of mitochondrial complex I (the whole enzyme complex)[8][6]. - The entire enzyme complex ("NADH-ubiquinone oxidoreductase", "complex I", or "respiratory complex I"), which is a large multi-subunit enzyme[3][4][1]. For most scientific or therapeutic targeting, the relevant entity is the entire NADH-ubiquinone oxidoreductase (complex I), not an individual subunit. If the intent is the subunit ND1, the canonical name should be "NADH-ubiquinone oxidoreductase chain 1 (ND1)"; if the intent is the whole enzyme, it should be "NADH-ubiquinone oxidoreductase (complex I)". The provided name "NADH dehydrogenase chain I" thus lacks specificity and does not follow accepted protein/gene nomenclature, and is flagged as potentially incorrect due to ambiguity and non-standard naming[6][8].

Other names
Respiratory complex INADH dehydrogenase (ubiquinone) chain IMitochondrial complex I subunit 1ND1
02

Mechanism of action

Inhibition of electron transport (e.g., by rotenone/piericidin A, which block electron transfer from NADH to ubiquinone) Modulation of mitochondrial reactive oxygen species (ROS) production

03

Biological functions

Mitochondrial electron transportOxidative phosphorylationProton translocationATP generation
04

Disease associations

Neurodegenerative diseaseCardiovascular diseaseMitochondrial disordersIschemia/reperfusion injuryParkinson's disease
05

Safety considerations

Mitochondrial toxicityRisk of lactic acidosis (for drugs that inhibit complex I, such as metformin in predisposed patients)Oxidative stress leading to cell damage
06

Interacting drugs

Metformin

2 more in the full profile.

07

Biomarkers

Complex I activity in muscle biopsy (proxy biomarker for mitochondrial diseases)Lactate/pyruvate ratio (for mitochondrial dysfunction)

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