TY  - JOUR
A1  - Kahlhöfer, Flora
A1  - Gansen, Max
A1  - Zickermann, Volker
T1  - 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
T2  - Life
N2  - 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.
KW  - mitochondrial disease
KW  - Leigh syndrome
KW  - NADH dehydrogenase
KW  - respiratory chain
KW  - oxidative phosphorylation
KW  - assembly factor
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/61313
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-613139
SN  - 2075-1729
VL  - 11
IS  - 5, art. 455
SP  - 1
EP  - 13
PB  - MDPI
CY  - Basel
ER  -