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
  • Leber hereditary optic neuropathy
  • Leber optic atrophy & dystonia
  • MELAS syndrome

Pharmacology

• investigational intravitreal gene therapy lenadogene nolparvovec (Lumevoq)

Laboratory

• MT-ND4 gene mutation

More general terms

• NADH dehydrogenase subunit
• flavoprotein

Component of

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

Database

• UniProt: http://www.uniprot.org/uniprot/P03905.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4538
• OMIM: https://mirror.omim.org/entry/500001
• OMIM: https://mirror.omim.org/entry/516003
• OMIM: https://mirror.omim.org/entry/535000
• OMIM: https://mirror.omim.org/entry/540000