NADH dehydrogenase 20 kD subunit, mitochondrial-encoded (complex 1-20KD, NADH ubiquinone oxidoreductase subunit FeS protein 7, NDUFS7, PSST subunit)

Function

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

Cofactor: binds 1 4Fe-4S cluster (putative)

Structure

belongs to the complex 1 20 kD subunit family

Compartment

mitochondria

Pathology

• defects in NDUFS7 are a cause of Leigh syndrome
• defects in NDUFS7 are a cause of NADH dehydrogenase deficiency

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/O75251.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:3742
• OMIM: https://mirror.omim.org/entry/252010
• OMIM: https://mirror.omim.org/entry/256000
• OMIM: https://mirror.omim.org/entry/601825