InterPro domain: IPR016668

General Information

  • Identifier IPR016668
  • Description NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
  • Number of genes 10
  • Gene duplication stats Loading...
  • Associated GO terms GO:0005747   GO:0006120  

Abstract

NDUFS6 is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis [ 1 ]. It harbours a Zn-binding site and is essential for biogenesis of mitochondrial complex I [ 2 ].


1. NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency. J. Clin. Invest. 114, 837-45
2. Accessory NUMM (NDUFS6) subunit harbors a Zn-binding site and is essential for biogenesis of mitochondrial complex I. Proc. Natl. Acad. Sci. U.S.A. 112, 5685-90

Species distribution

Gene table