Mitochondrial complex V expression and activity in cystinotic fibroblasts.

Abstract

Alterations in ATP metabolism have been proposed to be involved in the pathogenesis of cystinosis, the most common form of inherited Fanconi syndrome. A recent study showed normal activity of respiratory chain complexes I-IV with decreased ATP levels in cystinotic fibroblasts. Here, we show normal complex V expression and activity in mitochondria of cystinotic fibroblasts. This indicates that alterations in mitochondrial oxidative phosphorylation enzymes are not responsible for ATP decrease in cystinotic fibroblasts.

• Authors: Martijn J Wilmer, Lambertus P van den Heuvel, Richard J Rodenburg, Rutger O Vogel, Leo G Nijtmans, Leo A Monnens, Elena N Levtchenko
• Journal: Pediatric research (Pediatr Res) Vol. 64 Issue 5 Pg. 495-7 (Nov 2008) ISSN: 1530-0447 [Electronic] United States
• PMID: 18596576 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• complex V (mitochondrial oxidative phosphorylation system)
• Adenosine Triphosphate
• Mitochondrial Proton-Translocating ATPases

Topics

• Adenosine Triphosphate (metabolism)
• Blotting, Western
• Case-Control Studies
• Cells, Cultured
• Cystinosis (enzymology)
• Fibroblasts (enzymology)
• Humans
• Mitochondria (enzymology)
• Mitochondrial Proton-Translocating ATPases (metabolism)
• Oxidative Phosphorylation