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Mitochondrial complex V expression and activity in cystinotic fibroblasts

Martijn J Wilmer1, Lambertus P van den Heuvel, Richard J Rodenburg

  • 1Laboratory of Pediatrics and Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, 6500 HB, The Netherlands. m.wilmer@cukz.umcn.nl

Pediatric Research
|July 4, 2008
PubMed
Summary

Cystinosis, a cause of Fanconi syndrome, is linked to low ATP levels. This study found normal mitochondrial complex V activity, ruling out oxidative phosphorylation defects as the cause of decreased ATP in cystinotic cells.

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