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Biochemical characterization of muscle tissue of limb girdle muscular dystrophy: An 1 H and 13 C NMR study
Author(s) -
Sharma Uma,
Atri Surinder,
Sharma M. C.,
Sarkar Chitra,
Jagannathan N. R.
Publication year - 2003
Publication title -
nmr in biomedicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.278
H-Index - 114
eISSN - 1099-1492
pISSN - 0952-3480
DOI - 10.1002/nbm.832
Subject(s) - chemistry , glycolysis , metabolism , glutamine , nuclear magnetic resonance spectroscopy , lactic acid , biochemistry , medicine , muscular dystrophy , skeletal muscle , endocrinology , amino acid , biology , stereochemistry , bacteria , genetics
The metabolic differences between the muscle biopsies of patients with limb girdle muscular dystrophy (LGMD) and normal controls were characterized using high‐resolution 1 H and 13 C NMR spectroscopy. In all, 44 metabolites were unambiguously assigned in the perchloric acid extracts of skeletal muscle tissue, using 2D double quantum filtered (DQF COSY), total correlation (TOCSY), and 1 H/ 13 C heteronuclear multiple quantum coherence (HMQC) spectroscopy. The concentrations of glycolytic substrate, glucose ( p = 0.03), gluconeogenic amino acids, glutamine ( p = 0.02) and alanine ( p = 0.009) together with glycolytic product, lactate ( p = 0.04), were found to be significantly lowered in LGMD patients as compared with controls. The reduction in the concentration of glucose may be attributed to the decrease in the concentration of gluconeogenic amino acids in the degenerated muscle. Reduction in the rate of anaerobic glycolysis and lowered substrate concentration appear to be the possible reasons for the decrease in the concentration of lactate. A significant reduction in the concentration of choline in LGMD patients was also observed compared with controls. Lower concentration of choline may be the result of decreased rate of membrane turnover in LGMD patients. The data presented here provide an insight into the potentials of in‐vitro NMR spectroscopy in the study of muscle metabolism. Copyright © 2003 John Wiley & Sons, Ltd.