Muscle fatigue in McArdle's disease studied by 31P-NMR: Effect of glucose infusion

S. F. Lewis, R. G. Haller, J. D. Cook, R. L. Nunnally

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

In muscle phosphorylase deficiency (McArdle's disease) there is an abnormally rapid fatigue during strenuous exercise. Increasing substrate availability to working muscle can improve exercise tolerance but the effect on muscle energy metabolism has not been studied. Using phosphorus-31 nuclear magnetic resonance (31P-NMR) we examined forearm muscle ATP, phosphocreatine (PCr), inorganic phosphate (P(i)) and pH in a McArdle patient (MP) and two healthy subjects (HS) at rest and during intermittent maximal effort handgrip contractions under control conditions (CC) and during intravenous glucose infusion (GI). Under CC, MP gripped to impending forearm muscle contracture in 130 s with a marked decline in muscle PCr and a dramatic elevation in P(i). During GI MP exercised easily for >420 s at higher tensions and with attenuated PCr depletion and P(i) accumulation. In HS, muscle PCr and P(i) changed more modestly and were not affected by GI. In MP and HS, ATP changed little or not at all with exercise. The results suggest that alterations in the levels of muscle PCr and P(i) but not ATP are involved in the muscle fatigue in McArdle's disease and the improved exercise performance during glucose infusion.

Original languageEnglish (US)
Pages (from-to)1991-1994
Number of pages4
JournalJournal of Applied Physiology
Volume59
Issue number6
StatePublished - 1985

Fingerprint

Glycogen Storage Disease Type V
Muscle Fatigue
Phosphocreatine
Glucose
Muscles
Healthy Volunteers
Adenosine Triphosphate
Exercise
Forearm
Deficiency Diseases
Exercise Tolerance
Contracture
Intravenous Infusions
Phosphorus
Energy Metabolism
Fatigue
Magnetic Resonance Spectroscopy
Phosphates

ASJC Scopus subject areas

  • Endocrinology
  • Physiology
  • Orthopedics and Sports Medicine
  • Physical Therapy, Sports Therapy and Rehabilitation

Cite this

Muscle fatigue in McArdle's disease studied by 31P-NMR : Effect of glucose infusion. / Lewis, S. F.; Haller, R. G.; Cook, J. D.; Nunnally, R. L.

In: Journal of Applied Physiology, Vol. 59, No. 6, 1985, p. 1991-1994.

Research output: Contribution to journalArticle

Lewis, S. F. ; Haller, R. G. ; Cook, J. D. ; Nunnally, R. L. / Muscle fatigue in McArdle's disease studied by 31P-NMR : Effect of glucose infusion. In: Journal of Applied Physiology. 1985 ; Vol. 59, No. 6. pp. 1991-1994.
@article{816852a8d64f4846bd961539b290a90f,
title = "Muscle fatigue in McArdle's disease studied by 31P-NMR: Effect of glucose infusion",
abstract = "In muscle phosphorylase deficiency (McArdle's disease) there is an abnormally rapid fatigue during strenuous exercise. Increasing substrate availability to working muscle can improve exercise tolerance but the effect on muscle energy metabolism has not been studied. Using phosphorus-31 nuclear magnetic resonance (31P-NMR) we examined forearm muscle ATP, phosphocreatine (PCr), inorganic phosphate (P(i)) and pH in a McArdle patient (MP) and two healthy subjects (HS) at rest and during intermittent maximal effort handgrip contractions under control conditions (CC) and during intravenous glucose infusion (GI). Under CC, MP gripped to impending forearm muscle contracture in 130 s with a marked decline in muscle PCr and a dramatic elevation in P(i). During GI MP exercised easily for >420 s at higher tensions and with attenuated PCr depletion and P(i) accumulation. In HS, muscle PCr and P(i) changed more modestly and were not affected by GI. In MP and HS, ATP changed little or not at all with exercise. The results suggest that alterations in the levels of muscle PCr and P(i) but not ATP are involved in the muscle fatigue in McArdle's disease and the improved exercise performance during glucose infusion.",
author = "Lewis, {S. F.} and Haller, {R. G.} and Cook, {J. D.} and Nunnally, {R. L.}",
year = "1985",
language = "English (US)",
volume = "59",
pages = "1991--1994",
journal = "Journal of Applied Physiology",
issn = "0161-7567",
publisher = "American Physiological Society",
number = "6",

}

TY - JOUR

T1 - Muscle fatigue in McArdle's disease studied by 31P-NMR

T2 - Effect of glucose infusion

AU - Lewis, S. F.

AU - Haller, R. G.

AU - Cook, J. D.

AU - Nunnally, R. L.

PY - 1985

Y1 - 1985

N2 - In muscle phosphorylase deficiency (McArdle's disease) there is an abnormally rapid fatigue during strenuous exercise. Increasing substrate availability to working muscle can improve exercise tolerance but the effect on muscle energy metabolism has not been studied. Using phosphorus-31 nuclear magnetic resonance (31P-NMR) we examined forearm muscle ATP, phosphocreatine (PCr), inorganic phosphate (P(i)) and pH in a McArdle patient (MP) and two healthy subjects (HS) at rest and during intermittent maximal effort handgrip contractions under control conditions (CC) and during intravenous glucose infusion (GI). Under CC, MP gripped to impending forearm muscle contracture in 130 s with a marked decline in muscle PCr and a dramatic elevation in P(i). During GI MP exercised easily for >420 s at higher tensions and with attenuated PCr depletion and P(i) accumulation. In HS, muscle PCr and P(i) changed more modestly and were not affected by GI. In MP and HS, ATP changed little or not at all with exercise. The results suggest that alterations in the levels of muscle PCr and P(i) but not ATP are involved in the muscle fatigue in McArdle's disease and the improved exercise performance during glucose infusion.

AB - In muscle phosphorylase deficiency (McArdle's disease) there is an abnormally rapid fatigue during strenuous exercise. Increasing substrate availability to working muscle can improve exercise tolerance but the effect on muscle energy metabolism has not been studied. Using phosphorus-31 nuclear magnetic resonance (31P-NMR) we examined forearm muscle ATP, phosphocreatine (PCr), inorganic phosphate (P(i)) and pH in a McArdle patient (MP) and two healthy subjects (HS) at rest and during intermittent maximal effort handgrip contractions under control conditions (CC) and during intravenous glucose infusion (GI). Under CC, MP gripped to impending forearm muscle contracture in 130 s with a marked decline in muscle PCr and a dramatic elevation in P(i). During GI MP exercised easily for >420 s at higher tensions and with attenuated PCr depletion and P(i) accumulation. In HS, muscle PCr and P(i) changed more modestly and were not affected by GI. In MP and HS, ATP changed little or not at all with exercise. The results suggest that alterations in the levels of muscle PCr and P(i) but not ATP are involved in the muscle fatigue in McArdle's disease and the improved exercise performance during glucose infusion.

UR - http://www.scopus.com/inward/record.url?scp=0022339474&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022339474&partnerID=8YFLogxK

M3 - Article

C2 - 3865926

AN - SCOPUS:0022339474

VL - 59

SP - 1991

EP - 1994

JO - Journal of Applied Physiology

JF - Journal of Applied Physiology

SN - 0161-7567

IS - 6

ER -