Title: Mitochondrial complex V expression and activity in cystinotic fibroblasts
Authors: Wilmer, Martijn J ×
van den Heuvel, Lambertus
Rodenburg, Richard J
Vogel, Rutger O
Nijtmans, Leo G
Monnens, Leo A
Levtchenko, Elena #
Issue Date: Nov-2008
Publisher: Lippincott Williams & Wilkins
Series Title: Pediatric research vol:64 issue:5 pages:495-497
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.
ISSN: 0031-3998
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Section Child - Miscellaneous (-)
× corresponding author
# (joint) last author

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