ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics

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

Format: Article
Diterbitkan: Elsevier 2015-06-01

Deskripsi

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.