NADH dehydrogenase p75 (NADH ubiquinone oxidoreductase p75, NDUFS1, complex I-75kD, CI-75kD)

Function

• subunit of NADH dehydrogenase (mitochondrial complex 1)
• largest subunit of complex 1
• component of the iron-sulfur (IP) fragment of the enzyme

NADH + ubiquinone <--> NAD+ + ubiquinol

Cofactor:

• binds 1 2Fe-2S cluster per subunit (putative)
• binds 2 4Fe-4S clusters per subunit (putative)

Structure

• belongs to the complex 1 75 kD subunit family
• contains 1 2Fe-2S ferredoxin-type domain
• contains 1 4Fe-4S type ferredoxin domain

Compartment

mitochondrial inner membrane

Pathology

• defects in NDUFS1 are a cause of complex 1 mitochondrial respiratory chain 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/P28331.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4719
• OMIM: https://mirror.omim.org/entry/157655
• OMIM: https://mirror.omim.org/entry/252010