NADH dehydrogenase 51 kD subunit, mitochondrial-encoded (complex 1-51KD, NADH ubiquinone oxidoreductase flavoprotein 1, NDUFV1, UQOR1)

Function

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

Cofactor: binds 1 FMN (putative)

Structure

belongs to the complex 1 51 kD subunit family

Compartment

• mitochondrial inner membrane
• peripheral membrane protein, matrix side

Alternative splicing

named isoforms=2

Pathology

• defects in NDUFV1 are a cause of Leigh syndrome
• defects in NDUFV1 are a cause of NADH dehydrogenase

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/P49821.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4723
• OMIM: https://mirror.omim.org/entry/161015
• OMIM: https://mirror.omim.org/entry/252010
• OMIM: https://mirror.omim.org/entry/256000