Target intelligence / Profile preview

NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (NDUFS7)

Target
NDUFS7
Molecular classification
Enzyme, Mitochondrial respiratory chain complex subunit, Iron-sulfur protein
01

Overview

NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (NDUFS7), is a core subunit of Complex I of the mitochondrial respiratory chain, vital for electron transfer from NADH to ubiquinone, resulting in generation of a proton gradient used for ATP synthesis. It contains iron-sulfur clusters and is part of the catalytic core involved in electron transfer and proton translocation. Mutations in NDUFS7 cause mitochondrial complex I deficiency, especially Leigh syndrome—a severe neurodegenerative disorder—and other mitochondrial diseases. Dysfunction disrupts complex I assembly, increases oxidative stress, and impairs cellular respiration. NDUFS7 is not a direct therapeutic drug target but is of high clinical relevance in rare genetic and metabolic diseases.

Other names
NADH:ubiquinone oxidoreductase core subunit S7CI-20kDPSSTFLJ46880FLJ45860CI-20Complex I-20kDNADH-ubiquinone oxidoreductase 20 kDa subunitPSST subunitcomplex I 20kDa subunitNADH dehydrogenase (ubiquinone) Fe-S protein 720kDa (NADH-coenzyme Q reductase)MC1DN3MY017NADH:Ubiquinone Oxidoreductase Core Subunit S7NADH-Ubiquinone Oxidoreductase 20 KDa SubunitNADH Dehydrogenase (Ubiquinone) Fe-S Protein 7PSST SubunitComplex I 20kDa Subunit
02

Mechanism of action

Not applicable—no approved drugs directly targeting this subunit. Experimental modulation relates to complex I inhibition or mitochondrial function alteration, which generally affects electron transfer/proton translocation in NADH:ubiquinone oxidoreductase

03

Biological functions

Electron transport in mitochondrial respiratory chainOxidoreductase activityMetal ion (iron-sulfur cluster) bindingCell proliferation (indirectly, via mitochondrial function)Apoptosis (dysfunction leads to enhanced apoptosis)
04

Disease associations

Neurodegenerative disease (Leigh syndrome, Parkinson’s disease, Leber’s hereditary optic neuropathy)Mitochondrial disorders (Complex I deficiency, macrocephaly with progressive leukodystrophy, cardiomyopathy, myopathy, liver disease)
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Safety considerations

Therapeutic targeting could raise the risk of mitochondrial toxicity.Deficiency increases reactive oxygen species and cell deathClinical relevance is primarily as a genetic disease cause, not a pharmacological target.
06

Biomarkers

Mutations in NDUFS7 can be biomarkers for mitochondrial complex I deficiency and Leigh syndrome

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