title: Increasing fat in the diet does not improve muscle performance in patients with mitochondrial myopathy due to complex I deficiency.
abstract: Four myopathic patients with complex I deficiency followed diets containing 55 energy per cent (En%) as fat or 25 En% as fat, both for three weeks. Maximal workload and muscle force were not different on either diet. Exercise endurance time, oxygen consumption and lactate levels were also not different, but one patient had diminished endurance time on 25 En% as fat. Our observations do not support the use of increasing the fat in the diet of patients with mitochondrial complex I deficiency.
authors.affiliation: Department of Clinical Chemistry, VU University Medical Center, Amsterdam. k.demeer@vumc.nl
authors.forename: K
authors.initials: K
authors.lastname: de Meer
authors.forename: M J
authors.initials: MJ
authors.lastname: Roef
authors.forename: J B C
authors.initials: JB
authors.lastname: de Klerk
authors.forename: H D
authors.initials: HD
authors.lastname: Bakker
authors.forename: G P A
authors.initials: GP
authors.lastname: Smit
authors.forename: B T
authors.initials: BT
authors.lastname: Poll-The
mesh_terms: D000293:Adolescent; D000328:Adult; D003600:Cytosol / Q000378:metabolism; D004041:Dietary Fats / Q000627:therapeutic use*; D015444:Exercise; D005260:Female; D006801:Humans; D017240:Mitochondrial Myopathies / Q000097:blood* / Q000473:pathology*; D009132:Muscles / Q000473:pathology*; D009243:NAD / Q000378:metabolism; D010100:Oxygen / Q000378:metabolism; D010101:Oxygen Consumption; D013997:Time Factors
chemical_list: D004041:Dietary Fats; D009243:NAD; D010100:Oxygen