The role of pH on the thermodynamics and kinetics of muscle biochemistry: An in vivo study by 31P-MRS in patients with myo-phosphorylase deficiency

E. Malucelli, S. Iotti, D. N. Manners, C. Testa, A. Martinuzzi, B. Barbiroli, R. Lodi

Research output: Contribution to journalArticle

Abstract

In this study we assessed ΔG′ATP hydrolysis, cytosolic [ADP], and the rate of phosphocreatine recovery using Phosphorus Magnetic Resonance Spectroscopy in the calf muscle of a group of patients affected by glycogen myo-phosphorylase deficiency (McArdle disease). The goal was to ascertain whether and to what extent the deficit of the glycogenolytic pathway would affect the muscle energy balance. A typical feature of this pathology is the lack of intracellular acidosis. Therefore we posed the question of whether, in the absence of pH decrease, the rate of phosphocreatine recovery depends on the amount of phosphocreatine consumed during exercise. Results showed that at the end of exercise both [ADP] and ΔG′ATP of patients were significantly higher than those of matched control groups reaching comparable levels of phosphocreatine concentration. Furthermore, in these patients we found that the rate of phosphocreatine recovery is not influenced by the amount of phosphocreatine consumed during exercise. These outcomes provide experimental evidence that: i) the intracellular acidification occurring in exercising skeletal muscle is a protective factor for the energy consumption; and ii) the influence of pH on the phosphocreatine recovery rate is at least in part related to the kinetic mechanisms of mitochondrial creatine kinase enzyme.

Original languageEnglish
Pages (from-to)1244-1249
Number of pages6
JournalBiochimica et Biophysica Acta - Bioenergetics
Volume1807
Issue number9
DOIs
Publication statusPublished - Sep 2011

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Keywords

  • ΔG ATP
  • Energy metabolism
  • Glycogen
  • McArdle disease
  • Phosphorous magnetic resonance spectroscopy
  • Skeletal muscle

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Cell Biology

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