No effect of triheptanoin in patients with phosphofructokinase deficiency

•First study in PFKD to investigate fat and glucose metabolism with stable isotope techniques.•Triheptanoin treatment had no effect on heart rate or improvement of FAO during exercise.•Triheptanoin treatment increased plasma palmitate production during exercise. Phosphofructokinase deficiency (PFKD)...

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Veröffentlicht in:	Neuromuscular disorders : NMD 2022-04, Vol.32 (4), p.295-304
Hauptverfasser:	Raaschou-Pedersen, Daniel Emil, Madsen, Karen Lindhardt, Løkken, Nicoline, Storgaard, Jesper Helbo, Quinlivan, Ros, Laforêt, Pascal, Lund, Allan, Van Hall, Gerrit, Vissing, John, Ørngreen, Mette
Format:	Artikel
Sprache:	eng
Schlagworte:	Cross-Over Studies Endocrinology and metabolism fat and carbohydrate metabolism Glycogen Glycogen storage disease Human health and pathology Humans Life Sciences Metabolic Myopathies Palmitates Pharmaceutical sciences Pharmacology Phosphofructokinase deficiency Phosphofructokinases Pyruvates Triglycerides Triheptanoin
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