Effect of changes in fat availability on exercise capacity in McArdle disease

Archives of Neurology

Volumn 66, Issue 6, 2009, Pages 762-766

  Andersen, Susanne T ^a   Jeppesen, Tina D ^a   Taivassalo, Tanja ^b   Sveen, Marie Louise ^a   Heinicke, Katja ^b   Haller, Ronald G ^b,c   Vissing, John ^a,d  

^a UNIVERSITY OF COPENHAGEN   (Denmark)

^b Neuromuscular Center   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d Rigshospitalet   (Denmark)

Author keywords

[No Author keywords available]

Indexed keywords

CITRIC ACID; FATTY ACID; GLUCOSE; HEPARIN; INSULIN; INTRALIPID; LIPID; NICOTINIC ACID; SODIUM CHLORIDE;

ADULT; ARTICLE; CITRIC ACID CYCLE; CLINICAL ARTICLE; EXERCISE TOLERANCE; FATTY ACID BLOOD LEVEL; FATTY ACID OXIDATION; FEMALE; GLUCOSE BLOOD LEVEL; GLUCOSE INFUSION; GLYCOGEN MUSCLE LEVEL; GLYCOGEN STORAGE DISEASE TYPE 5; GLYCOGENOLYSIS; HEART RATE; HOSPITAL CARE; HUMAN; LIPID METABOLISM; LIPOLYSIS; MALE; METABOLITE; OUTCOME ASSESSMENT; PRIORITY JOURNAL; WORKLOAD;

ADIPOSE TISSUE; ADULT; ANTILIPEMIC AGENTS; CITRIC ACID CYCLE; CROSS-OVER STUDIES; ENERGY METABOLISM; EXERCISE TOLERANCE; FATTY ACIDS, NONESTERIFIED; FEMALE; GLYCOGEN STORAGE DISEASE TYPE V; GLYCOLYSIS; HUMANS; LIPID MOBILIZATION; MALE; MUSCLE STRENGTH; MUSCLE, SKELETAL; NIACIN; OXYGEN CONSUMPTION; PHYSICAL FITNESS; PLACEBOS; TREATMENT OUTCOME; YOUNG ADULT;

EID: 67049115835     PISSN: 00039942     EISSN: 15383687     Source Type: Journal    
DOI: 10.1001/archneurol.2009.93     Document Type: Article

References (29)

1. Vissing J, Haller RG. Metabolic myopathies. In: Pourmand R, ed. Neuromuscular Diseases: Expert Clinicians' Views. Boston, MA: Butterworth-Heinemann; 2001:393-410.
2. Andersen ST, Dunø M, Schwartz M, Vissing J. Do carriers of PYGM mutations have symptoms of McArdle disease? Neurology. 2006;67(4):716-718. (Pubitemid 44273632)
3. Vissing J, Lewis SF, Galbo H, Haller RG. Effect of deficient muscular glycogenolysis on extramuscular fuel production in exercise. J Appl Physiol. 1992;72(5):1773-1779.
4. Spencer MK, Katz A. Role of glycogen in control of glycolysis and IMP formation in human muscle during exercise. Am J Physiol. 1991;260(6, pt 1):E859-E864.
5. Spencer MK, Yan Z, Katz A. Effect of low glycogen on carbohydrate and energy metabolism in human muscle during exercise. Am J Physiol. 1992;262(4, pt 1):C975-C979.
6. Haller RG, Vissing J. Spontaneous "second wind" and glucose-induced second "second wind" in McArdle disease: oxidative mechanisms. Arch Neurol. 2002;59(9):1395-1402.
7. Koh HJ, Hirshman MF, He H, et al. Adrenaline is a critical mediator of acute exercise-induced AMP-activated protein kinase activation in adipocytes. Biochem J. 2007;403(3):473-481. (Pubitemid 46706975)
8. Ørngreen MC, Jeppesen TD, Andersen ST, et al. Fat metabolism during exercise in patients with McArdle disease. Neurology. 2009;72(8):718-724.
9. Karpe F, Frayn KN. The nicotinic acid receptor: a new mechanism for an old drug. Lancet. 2004;363(9424):1892-1894. (Pubitemid 38748647)
10. Lutz EP, Merkel M, Kako Y, et al. Heparin-binding defective lipoprotein lipase is instable and causes abnormalities in lipid delivery to tissues. J Clin Invest. 2001;107(9):1183-1192. (Pubitemid 32422915)
11. Romijn JA, Coyle EF, Sidossis LS, et al. Regulation of endogenous fat and carbohydrate metabolism in relation to exercise intensity and duration. Am J Physiol. 1993;265(3, pt 1):E380-E391. (Pubitemid 23296552)
12. van Loon LJ, Greenhaff PL, Constantin-Teodosiu D, Saris WH, Wagenmakers AJ. The effect of increasing exercise intensity on muscle fuel utilisation in humans. J Physiol. 2001;536(pt 1):295-304. (Pubitemid 32952238)
13. Astrand PO, Rodahl K, Dahl HA, Stromme SB. Textbook of Work Physiology: Physiological Bases of Exercise. Champaign, IL: Human Kinetics Inc; 2003:369-394.
14. Boden G. Role of fatty acids in the pathogenesis of insulin resistance and NIDDM [published correction appears in Diabetes. 1997;46(3):536]. Diabetes. 1997;46(1):3-10.
15. Randle PJ. Regulatory interactions between lipids and carbohydrates: the glucose fatty acid cycle after 35 years. Diabetes Metab Rev. 1998;14(4):263-283. (Pubitemid 29110250)
16. Storgaard H, Jensen CB, Vaag AA, Vølund A, Madsbad S. Insulin secretion after short- and long-term low-grade free fatty acid infusion in men with increased risk of developing type 2 diabetes. Metabolism. 2003;52(7):885-894. (Pubitemid 36949553)
17. Sahlin K, Jorfeldt L, Henriksson KG, Lewis SF, Haller RG. Tricarboxylic acid cycle intermediates during incremental exercise in healthy subjects and in patients with McArdle's disease. Clin Sci (Lond). 1995;88(6):687-693.
18. Sahlin K, Katz A, Broberg S. Tricarboxylic acid cycle intermediates in human muscle during prolonged exercise [published corrections appear in Am J Physiol. 1995;268(2, pt 1) and Am J Physiol. 1995;268(6, pt 3)]. Am J Physiol. 1990;259(5, pt 1):C834-C841.
19. Lee SH, Davis EJ. Carboxylation and decarboxylation reactions: anaplerotic flux and removal of citrate cycle intermediates in skeletal muscle. J Biol Chem. 1979;254(2):420-430.
20. Williamson JR, Cooper RH. Regulation of the citric acid cycle in mammalian systems. FEBS Lett. 1980;117(suppl):K73-K85.
21. Graham TE, Gibala MJ. Anaplerosis of the tricarboxylic acid cycle in human skeletal muscle during exercise. In: Richter EA, Kiens B, Galbo H, eds, et al. Skeletal Muscle Metabolism in Exercise and Diabetes. New York, NY: Plenum Press; 2007:271-286.
22. Vissing J, Haller RG. A diagnostic cycle test for McArdle's disease. Ann Neurol. 2003;54(4):539-542. (Pubitemid 37176554)
23. Carroll JE, DeVivo DC, Brooke MH, Planer GJ, Hagberg JH. Fasting as a provocative test in neuromuscular diseases. Metabolism. 1979;28(6):683-687. (Pubitemid 9187452)
24. Lewis SF, Haller RG, Cook JD, Blomqvist CG. Metabolic control of cardiac output response to exercise in McArdle's disease. J Appl Physiol. 1984;57(6):1749-1753. (Pubitemid 15191372)
25. Haller RG, Lewis SF. Abnormal ventilation during exercise in McArdle's syndrome: modulation by substrate availability. Neurology. 1986;36(5):716-719. (Pubitemid 16074248)
26. Busch V, Gempel K, Hack A, et al. Treatment of glycogenosis type V with ketogenic diet [letter]. Ann Neurol. 2005;58(2):341. (Pubitemid 41098895)
27. Viskoper RJ, Wolf E, Chaco J, Katz R, Chowers I. McArdle's syndrome: the reaction to a fat-rich diet. Am J Med Sci. 1975;269(2):217-221.
28. Vissing J, Quistorff B, Haller RG. Effect of fuels on exercise capacity in muscle phosphoglycerate mutase deficiency. Arch Neurol. 2005;62(9):1440-1443. (Pubitemid 41352062)
29. Haller RG, Lewis SF. Glucose-induced exertional fatigue in muscle phosphofructokinase deficiency. N Engl J Med. 1991;324(6):364-369.