Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis.

ABSTRACT: As clinical evidence supports a negative impact of dysfunctional energy metabolism on the disease progression in amyotrophic lateral sclerosis, it is vital to understand how the energy metabolic pathways are altered and whether they can be restored to slow disease progression. Possible approaches include increasing or rerouting catabolism of alternative fuel sources to supplement the glycolytic and mitochondrial pathways such as glycogen, ketone bodies and nucleosides. To analyse the basis of the catabolic defect in amyotrophic lateral sclerosis we used a novel phenotypic metabolic array. We profiled fibroblasts and induced neuronal progenitor-derived human induced astrocytes from C9orf72 amyotrophic lateral sclerosis patients compared to normal controls, measuring the rates of production

SUBMITTER: Allen SP 

PROVIDER: S-EPMC6391613 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature