NADH dehydrogenase 23 kD subunit; mitochondrial-encoded NADH dehydrogenase [ubiquinone] iron-sulfur protein 8; complex 1-42KD; CI-23kD; TYKY subunit (NDUFS8)

Function

• core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex 1)
• component of minimal assembly required for catalysis

Cofactor: binds 2 4Fe-4S clusters per subunit (putative)

Structure

• belongs to the complex 1 23 kD subunit family
• contains 2 4Fe-4S type ferredoxin domains

Compartment

mitochondria (probable)

Pathology

defects in NDUFS8 are a cause of Leigh syndrome

More general terms

• NADH dehydrogenase subunit

Component of

• NADH dehydrogenase; respiratory chain complex-I; NADH CoQ reductase;NADH-ubiquinone oxidoreductase

Database

• UniProt: http://www.uniprot.org/uniprot/O00217.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4728
• OMIM: https://mirror.omim.org/entry/256000
• OMIM: https://mirror.omim.org/entry/602141