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NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 (NDUFA8)

Target: NDUFA8
Molecular classification: Enzyme (component of NADH dehydrogenase [ubiquinone]/Complex I), Accessory subunit of mitochondrial membrane respiratory chain Complex I

Overview

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 (NDUFA8) is a nuclear-encoded accessory protein of mitochondrial Complex I, the largest component of the electron transport chain in the inner mitochondrial membrane. This protein aids in transferring electrons from NADH to ubiquinone, a process fundamental to cellular ATP production. NDUFA8 is not directly involved in catalysis but is essential for proper assembly and stability of Complex I. Mutations in NDUFA8 are linked to mitochondrial pathologies including Leigh syndrome, cancer, and diabetes mellitus, highlighting its physiological and clinical importance[1][2][8][9].

Other names: NDUFA8NADH dehydrogenase 1 alpha subcomplex subunit 8NADH:ubiquinone oxidoreductase subunit A8

Biological functions

Electron transfer from NADH to ubiquinone in the mitochondrial inner membrane (oxidative phosphorylation)Cellular energy production (ATP synthesis via respiratory chain)

Disease associations

Mitochondrial disorders (Complex I deficiency, Leigh syndrome, infantile-onset encephalopathies)Cancer (as part of mitochondrial regulation; mutations associated, but specific role poorly understood)Diabetes mellitus (implicated in energy metabolism, pathogenic variants associated)

Safety considerations

Mitochondrial dysfunction (toxicity risks with Complex I inhibition; mutations cause severe disease phenotypes)Energetic failure in tissues with high metabolic demand

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