C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis-Reference-Cited by-同舟云学术

C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis

Published:2019-10-24 Issue:12 Volume:142 Page:3771-3790
ISSN:0006-8950
Container-title:Brain
language:en
Short-container-title:

Author:

Allen Scott P¹^ORCID,Hall Benjamin¹,Woof Ryan¹,Francis Laura²,Gatto Noemi¹^ORCID,Shaw Allan C¹,Myszczynska Monika¹,Hemingway Jordan¹,Coldicott Ian¹,Willcock Amelia¹,Job Lucy¹,Hughes Rachel M¹,Boschian Camilla¹,Bayatti Nadhim¹,Heath Paul R¹,Bandmann Oliver¹,Mortiboys Heather¹,Ferraiuolo Laura¹,Shaw Pamela J¹

Affiliation:

1. Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385 Glossop Road, Sheffield S10 2HQ, UK

2. The Living Systems Institute, University of Exeter, Stocker Road, Exeter, EX4 4QD, UK

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.

Funder

Motor Neurone Disease Association Senior Fellowship

Medical Research Council

MND Association

NIHR Senior Investigator

Academy of Medical Sciences

Sheffield NIHR Biomedical Research Centre

Parkinson’s UK

Publisher

Oxford University Press (OUP)

Subject

Clinical Neurology