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C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis

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Energy metabolism is altered in amyotrophic lateral sclerosis and its animal models. Using metabolic profiling, Allen et al. reveal a loss of metabolic flexibility in induced astrocytes derived from patients… Click to show full abstract

Energy metabolism is altered in amyotrophic lateral sclerosis and its animal models. Using metabolic profiling, Allen et al. reveal a loss of metabolic flexibility in induced astrocytes derived from patients with C9orf72 ALS, caused by defects in glycogen, fructose and mitochondrial energy substrate transport.

Keywords: amyotrophic lateral; metabolic flexibility; lateral sclerosis; c9orf72 expansion

Journal Title: Brain
Year Published: 2019

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