Impairment of sympathetic activation during static exercise in patients with muscle phosphorylase deficiency (McArdle's disease)
| Autor: | Ronald G. Haller, Susan L. Pryor, L. A. Bertocci, Steven F. Lewis, Ronald G. Victor |
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| Rok vydání: | 1990 |
| Předmět: |
Adult
medicine.medical_specialty Sympathetic nervous system Magnetic Resonance Spectroscopy Sympathetic Nervous System Glycogenolysis Phosphocreatine Phosphorylases Physical Exertion Phosphates Reference Values Internal medicine medicine Humans Myocyte Chemistry Muscles Skeletal muscle General Medicine Microneurography Hydrogen-Ion Concentration medicine.disease Adenosine Diphosphate Endocrinology medicine.anatomical_structure Myophosphorylase Reflex Glycogen Storage Disease Type V Glycogen storage disease type V Research Article |
| Zdroj: | Journal of Clinical Investigation. 85:1444-1449 |
| ISSN: | 0021-9738 |
| DOI: | 10.1172/jci114589 |
| Popis: | 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 pressor 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. |
| Databáze: | OpenAIRE |
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