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NADH dehydrogenase subunit-4

Function: - subunit of NADH dehydrogenase (mitochondrial complex 1) NADH + ubiquinone NAD+ + ubiquinol Structure: belongs to the complex 1 subunit 4 family Compartment: mitochondrial inner membrane Pathology: - defects in MT-ND4 are a cause of a) Leber hereditary optic neuropathy b) Leber optic atrophy & dystonia c) MELAS syndrome Laboratory: - MT-ND4 gene mutation Pharmacology: - investigational intravitreal gene therapy lenadogene nolparvovec (Lumevoq)

Related

NADH dehydrogenase subunit-4 gene

General

flavoprotein NADH dehydrogenase subunit

Properties

MOTIF: cofactor-binding site COFACTOR-BOUND: flavin

Database Correlations

OMIM correlations UniProt P03905 PFAM correlations Kegg hsa:4538 ENZYME 1.6.5.3

References

  1. UniProt :accession P03905
  2. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=MT-ND4

Component-of

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