Molecular Genetics and Metabolism Reports (Jun 2015)

ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics

  • Dolly Prabhu,
  • Amy C. Goldstein,
  • Riyad El-Khoury,
  • Malgorzata Rak,
  • Lia Edmunds,
  • Pierre Rustin,
  • Jerry Vockley,
  • Manuel Schiff

DOI
https://doi.org/10.1016/j.ymgmr.2015.03.005
Journal volume & issue
Vol. 3, no. C
pp. 43 – 46

Abstract

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Adenine nucleotide translocase 2 (ANT2) transports glycolytic ATP across the inner mitochondrial membrane. Patients with ANT2 deletion were recently reported. We aimed at characterizing mitochondrial functions in ANT2-defective fibroblasts. In spite of ANT2 expression in fibroblasts, we observed no difference between ANT2-defective and control fibroblasts for mitochondrial respiration, respiratory chain activities, mitochondrial membrane potential and intracellular ATP levels. This indicates that ANT2 insufficiency does not alter fibroblast basal mitochondrial bioenergetics.

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