Impairment of sympathetic activation during static exercise in patients with muscle phosphorylase deficiency (McArdle's disease)

Susan L. Pryor, Steven F. Lewis, Ronald G. Haller, Loren A. Bertocci, Ronald G. Victor

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Static exercise in normal humans causes reflex increases in muscle sympathetic nerve activity (MSNA) that are closely coupled to the contraction-induced decrease in muscle cell pH, an index of glycogen degradation and glycolytic flux. To determine if sympathetic activation is attenuated when muscle glycogenolysis is blocked due to myophosphorylase deficiency (McArdle's disease), an inborn enzymatic defect localized to skeletal muscle, we now have performed microelectrode recordings of MSNA in four patients with McArdle's disease during static handgrip contraction. A level of static handgrip that more than doubled MSNA in normal humans had no effect on MSNA and caused an attenuated rise in blood pressure in the patients with myophosphorylase deficiency. In contrast, two nonexercise sympathetic stimuli, Valsalva's maneuver and cold presser stimulation, evoked comparably large increases in MSNA in patients and normals. The principal new conclusion is that defective glycogen degradation in human skeletal muscle is associated with a specific reflex impairment in sympathetic activation during static exercise.

Original languageEnglish (US)
Pages (from-to)1444-1449
Number of pages6
JournalJournal of Clinical Investigation
Volume85
Issue number5
StatePublished - May 1990

Fingerprint

Glycogen Storage Disease Type V
Deficiency Diseases
Exercise
Muscles
Glycogen
Reflex
Skeletal Muscle
Glycogenolysis
Valsalva Maneuver
Microelectrodes
Muscle Cells
Blood Pressure

Keywords

  • P nuclear magnetic resonance spectroscopy
  • Microneurography
  • Myophosphorylase deficiency
  • Sympathetic nerve activity

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Impairment of sympathetic activation during static exercise in patients with muscle phosphorylase deficiency (McArdle's disease). / Pryor, Susan L.; Lewis, Steven F.; Haller, Ronald G.; Bertocci, Loren A.; Victor, Ronald G.

In: Journal of Clinical Investigation, Vol. 85, No. 5, 05.1990, p. 1444-1449.

Research output: Contribution to journalArticle

Pryor, Susan L. ; Lewis, Steven F. ; Haller, Ronald G. ; Bertocci, Loren A. ; Victor, Ronald G. / Impairment of sympathetic activation during static exercise in patients with muscle phosphorylase deficiency (McArdle's disease). In: Journal of Clinical Investigation. 1990 ; Vol. 85, No. 5. pp. 1444-1449.
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