The human complex I NDUFS4 subunit: from gene structure to function and pathology

S M S Budde; L P W J van den Heuvel; J A M Smeitink

doi:10.1016/s1567-7249(02)00035-1

The human complex I NDUFS4 subunit: from gene structure to function and pathology

Mitochondrion. 2002 Nov;2(1-2):109-15. doi: 10.1016/s1567-7249(02)00035-1.

Authors

S M S Budde¹, L P W J van den Heuvel, J A M Smeitink

Affiliation

¹ Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Geert Grooteplein 20, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.

PMID: 16120313
DOI: 10.1016/s1567-7249(02)00035-1

Abstract

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.