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MGP002069

UniProt Annotations

Entry Information
Gene NameNADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
Protein EntryNDUS8_HUMAN
UniProt IDO00217
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityNADH + acceptor = NAD(+) + reduced acceptor.
Catalytic ActivityNADH + ubiquinone + 5 H(+)(In) = NAD(+) + ubiquinol + 4 H(+)(Out).
CofactorName=[4Fe-4S] cluster; Xref=ChEBI:CHEBI:49883; Evidence={ECO:0000250}; Note=Binds 2 [4Fe-4S] clusters per subunit. {ECO:0000250};
DiseaseLeigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia. {ECO:0000269|PubMed:9837812}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionCore subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). May donate electrons to ubiquinone. {ECO:0000250}.
SimilarityBelongs to the complex I 23 kDa subunit family. {ECO:0000305}.
SimilarityContains 2 4Fe-4S ferredoxin-type domains. {ECO:0000305}.
Subcellular LocationMitochondrion {ECO:0000305}.
SubunitMammalian complex I is composed of 45 different subunits. {ECO:0000269|PubMed:12611891}.
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