Accessory subunits of the matrix arm of mitochondrial complex I with a focus on subunit NDUFS4 and its role in complex I function and assembly

  • NADH:ubiquinone-oxidoreductase (complex I) is the largest membrane protein complex of the respiratory chain. Complex I couples electron transfer to vectorial proton translocation across the inner mitochondrial membrane. The L shaped structure of complex I is divided into a membrane arm and a matrix arm. Fourteen central subunits are conserved throughout species, while some 30 accessory subunits are typically found in eukaryotes. Complex I dysfunction is associated with mutations in the nuclear and mitochondrial genome, resulting in a broad spectrum of neuromuscular and neurodegenerative diseases. Accessory subunit NDUFS4 in the matrix arm is a hot spot for mutations causing Leigh or Leigh-like syndrome. In this review, we focus on accessory subunits of the matrix arm and discuss recent reports on the function of accessory subunit NDUFS4 and its interplay with NDUFS6, NDUFA12, and assembly factor NDUFAF2 in complex I assembly.
Author:Flora Kahlhöfer, Max Gansen, Volker ZickermannORCiDGND
Parent Title (English):Life
Place of publication:Basel
Document Type:Article
Date of Publication (online):2021/05/19
Date of first Publication:2021/05/19
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2021/06/30
Tag:Leigh syndrome; NADH dehydrogenase; assembly factor; mitochondrial disease; oxidative phosphorylation; respiratory chain
Issue:5, art. 455
Page Number:13
First Page:1
Last Page:13
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - Namensnennung 4.0