Assembling complex I with ACAD9

Cell Metab. 2010 Sep 8;12(3):211-2. doi: 10.1016/j.cmet.2010.08.008.

Abstract

Acyl-Co dehydrogenase 9 (ACAD9) was thought to play a role in fatty acid oxidation. Nouws et al. (2010) reveal a novel and essential role for this enzyme in mitochondrial complex I assembly. A mutation in ACAD9 causes an isolated complex I deficiency in a subset of patients with mitochondrial disease.

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