Mechanisms of exertional fatigue in muscle glycogenoses

John Vissing, Ronald G. Haller

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Exertional fatigue early in exercise is a clinical hallmark of muscle glycogenoses, which is often coupled with painful muscle contractures and episodes of myoglobinuria. A fundamental biochemical problem in these conditions is the impaired generation of ATP to fuel muscle contractions, which relates directly to the metabolic defect, but also to substrate-limited energy deficiency, as exemplified by the " second wind" phenomenon in McArdle disease. A number of secondary events may also play a role in inducing premature fatigue in glycogenoses, including (1) absent or blunted muscle acidosis, which may be important for maintaining muscle membrane excitability by decreasing chloride permeability, (2) loss of the osmotic effect related to lactate accumulation, which may account for absence of the normal increase in water content of exercised muscle, and thus promote higher than normal concentrations of extracellular potassium in exercising muscle and (3) exaggerated accumulation of ADP during exercise that may inhibit sodium-potassium and calcium-ATPases. Disorders of muscle glycogenolysis and glycolysis reveal the crucial role of these metabolic processes for supplying both anaerobic and aerobic energy for muscle contraction; and the pathological fatigue that occurs when glycogenolysis and/or glycolysis is blocked imply an important role for theses metabolic pathways in normal muscle fatigue.

Original languageEnglish (US)
JournalNeuromuscular Disorders
Volume22
Issue numberSUPPL. 3
DOIs
StatePublished - Dec 1 2012

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Glycogen Storage Disease
Muscle Fatigue
Muscles
Fatigue
Glycogenolysis
Glycolysis
Muscle Contraction
Potassium
Glycogen Storage Disease Type V
Glycogen Storage Disease Type I
Myoglobinuria
Contracture
Muscular Diseases
Acidosis
Metabolic Networks and Pathways
Adenosine Diphosphate
Chlorides
Permeability
Lactic Acid
Adenosine Triphosphate

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Genetics(clinical)
  • Neurology

Cite this

Mechanisms of exertional fatigue in muscle glycogenoses. / Vissing, John; Haller, Ronald G.

In: Neuromuscular Disorders, Vol. 22, No. SUPPL. 3, 01.12.2012.

Research output: Contribution to journalArticle

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