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The protein encoded by NDUFA4 belongs to the complex I 9kDa subunit family.
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Findings support reassignment of the NDUFA4 protein to complex IV and suggest that patients with unexplained COX deficiency should be screened for NDUFA4 mutations.
The protein encoded by this gene belongs to the complex I 9kDa subunit family. Mammalian complex I of mitochondrial respiratory chain is composed of 45 different subunits. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Complex I 9kDa subunit
, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4
, NADH-ubiquinone oxidoreductase MLRQ subunit
, complex I-MLRQ
, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4 (9 kDa, MLRQ)
, NADH dehydrogenase ubiquinone 1 alpha subcomplex 4
, mitochondrial complex I subunit NDUFA4