title: Effect of diet on exercise tolerance in carnitine palmitoyltransferase II deficiency.
abstract: It is generally believed that a diet high in carbohydrate improves exercise tolerance in patients with carnitine palmitoyltransferase II (CPT II) deficiency, but it has never been systematically investigated. The authors investigated the effect of a high- vs low-carbohydrate diet on exercise tolerance in four patients with CPT II, who cycled at a constant workload of 50% of VO2max. Exercise tolerance, assessed by exercise duration and perceived exertion, improved on the carbohydrate-rich diet.
authors.affiliation: Copenhagen Muscle Research Center and Department of Neurology, National University Hospital, Rigshospitalet, Denmark.
authors.forename: Mette C
authors.initials: MC
authors.lastname: Ørngreen
authors.forename: Rasmus
authors.initials: R
authors.lastname: Ejstrup
authors.forename: John
authors.initials: J
authors.lastname: Vissing
mesh_terms: D000409:Alanine / Q000097:blood; D001786:Blood Glucose / Q000032:analysis; D002334:Carnitine O-Palmitoyltransferase / Q000172:deficiency*; D003402:Creatine Kinase / Q000097:blood; D052283:Creatine Kinase, MM Form; D004040:Dietary Carbohydrates / Q000378:metabolism / Q000627:therapeutic use*; D004041:Dietary Fats / Q000378:metabolism; D005080:Exercise Test; D017079:Exercise Tolerance; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005990:Glycerol / Q000097:blood; D006003:Glycogen / Q000378:metabolism; D006339:Heart Rate; D006801:Humans; D007328:Insulin / Q000097:blood; D007527:Isoenzymes / Q000097:blood; D007773:Lactates / Q000097:blood; D008052:Lipid Metabolism, Inborn Errors / Q000097:blood / Q000150:complications / Q000178:diet therapy* / Q000235:genetics; D009135:Muscular Diseases / Q000097:blood / Q000178:diet therapy* / Q000235:genetics; D009638:Norepinephrine / Q000097:blood
chemical_list: D001786:Blood Glucose; D004040:Dietary Carbohydrates; D004041:Dietary Fats; D005230:Fatty Acids, Nonesterified; D007328:Insulin; D007527:Isoenzymes; D007773:Lactates; D006003:Glycogen; D002334:Carnitine O-Palmitoyltransferase; D003402:Creatine Kinase; D052283:Creatine Kinase, MM Form; D000409:Alanine; D005990:Glycerol; D009638:Norepinephrine