ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics
Dolly Prabhu, Amy Goldstein, Riyad El‐Khoury, Malgorzata Rak, Lia R. Edmunds, Pierre Rustin, Jerry Vockley, Manuel Schiff
Abstract
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Dolly Prabhu, Amy Goldstein, Riyad El‐Khoury, Malgorzata Rak, Lia R. Edmunds, Pierre Rustin, Jerry Vockley, Manuel Schiff
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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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.
Key concepts: Bioenergetics, ATP–ADP translocase, Mitochondrion, Respiration, Intracellular, Mitochondrial carrier, Biology, Fibroblast