ALS Compendium

Title: Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels

Year Published: 2013

Observation: VCP deficiency is associated with decreased mitochondrial membrane potential and decreased ATP levels. Oxygen consumption is increased in VCP deficient cells

Comment: Decreased ATP levels in VCP-deficient cells lower their energy capacity, making them more vulnerable to high energy-demanding processes such as ischemia

Citation: Bartolome, F., Wu, H.-C., Burchell, V. S., Preza, E., Wray, S., Mahoney, C. J., … Plun-Favreau, H. (2013). Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels. Neuron, 78(1), 57–64. doi:10.1016/j.neuron.2013.02.028

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