Target intelligence / Profile preview

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (NDUFS3)

Target
NDUFS3
Molecular classification
Enzyme, Mitochondrial respiratory chain protein, Iron-sulfur protein, Oxidoreductase
01

Overview

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (NDUFS3) is a core subunit of mitochondrial respiratory chain Complex I (NADH:ubiquinone oxidoreductase)[6][2][3][1]. It functions in electron transfer from NADH to ubiquinone, which is central to generating the proton gradient used for ATP synthesis through oxidative phosphorylation. NDUFS3 is essential for both the catalytic activity and the assembly of Complex I[3][6][4]. Mutations in this gene are associated with mitochondrial complex I deficiency and Leigh syndrome, showing its critical role in cellular energy metabolism and health[2][4][5]. It has emerging links to cancer cell proliferation via its role in glucose metabolism and mitochondrial function[4]. No drugs directly target NDUFS3 for therapy, but impairment can influence response to mitochondrial toxins and may serve as a diagnostic or disease-monitoring biomarker in select contexts.

Other names
NADH:ubiquinone oxidoreductase core subunit S3NDUFS3CI-30kDCI-30Complex I-30kDComplex I 30kDa subunitNADH-ubiquinone oxidoreductase 30 kDa subunitNADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDaNADH-coenzyme Q reductase
02

Mechanism of action

Electron transfer from NADH to ubiquinone in the mitochondrial respiratory chain (drugs targeting this process would affect ATP generation and mitochondrial function) Modulation by redox-active or mitochondrial-targeted drugs, if any (specific clinical modulators not established)

03

Biological functions

Mitochondrial electron transportCellular respirationAerobic ATP synthesisOxidoreductase activityComplex I assembly in the respiratory chain
04

Disease associations

Mitochondrial disease (e.g., mitochondrial complex I deficiency)Neurodegenerative disease (e.g., Leigh syndrome)Cancer (implicated in proliferation and metabolic reprogramming)Cardiovascular disease (if complex I is broadly impaired)
05

Safety considerations

Inhibition or dysfunction leads to severe mitochondrial disorders, such as Leigh syndrome and complex I deficiencyMitochondrial toxicity and resulting energetic failure potential with non-selective mitochondrial inhibition
06

Interacting drugs

Doxorubicin (substrate/enzyme interaction; not a direct inhibitor or modulator)

1 more in the full profile.

07

Biomarkers

NDUFS3 mutation/deletion as biomarker for mitochondrial complex I deficiency or Leigh syndromeExpression levels may serve as a biomarker for mitochondrial function or dysfunction in research

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