Article
Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type IIIa are phenotype-specific: An investigator-initiated, randomized, crossover study.
Journal of inherited metabolic disease - 1 Jan 2021
Hoogeveen Irene J, de Boer Foekje, Boonstra Willemijn F, van der Schaaf Caroline J, Steuerwald Ulrike, Sibeijn-Kuiper Anita J, Vegter Riemer J K, van der Hoeven Johannes H, Heiner-Fokkema M Rebecca, Clarke Kieran C, Cox Pete J, Derks Terry G J, Jeneson Jeroen A L
Abstract excerpt
Glycogen storage disease type IIIa (GSDIIIa) is an inborn error of carbohydrate metabolism caused by a debranching enzyme deficiency. A subgroup of GSDIIIa patients develops severe myopathy. The purpose of this study was to investigate whether acute nutritional ketosis (ANK) in response to ketone-ester (KE) ingestion is effective to deliver oxidative substrate to exercising muscle in GSDIIIa patients. This was an...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.