The human complex I NDUFS4 subunit: from gene structure to function and pathology.
Complex I is the first and largest enzyme of the oxidative phosphorylation system. It consists of at least 43 subunits. Recent studies have shown that the NDUFS4 subunit of complex I contributes to the activation of the complex through cAMP dependent phosphorylation of a conserved site (RVS) located at the C-terminal region of this protein. This report focuses on the NDUFS4 subunit. Summarized is the current knowledge of this subunit, from gene structure to function and pathology.
Budde
S M S
SM
Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Geert Grooteplein 20, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
van den Heuvel
L P W J
LP
Smeitink
J A M
JA