title: Biochemical characterization of muscle tissue of limb girdle muscular dystrophy: an 1H and 13C NMR study.
abstract: The metabolic differences between the muscle biopsies of patients with limb girdle muscular dystrophy (LGMD) and normal controls were characterized using high-resolution 1H and 13C 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 1H/13C 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.
authors.affiliation: Department of NMR, All India Institute of Medical Sciences, New Delhi-110 029, India.
authors.forename: Uma
authors.initials: U
authors.lastname: Sharma
authors.forename: Surinder
authors.initials: S
authors.lastname: Atri
authors.forename: M C
authors.initials: MC
authors.lastname: Sharma
authors.forename: Chitra
authors.initials: C
authors.lastname: Sarkar
authors.forename: N R
authors.initials: NR
authors.lastname: Jagannathan
mesh_terms: D000596:Amino Acids / Q000378:metabolism; D000818:Animals; D002462:Cell Membrane / Q000737:chemistry; D005947:Glucose / Q000378:metabolism; D006801:Humans; D019344:Lactic Acid / Q000378:metabolism; D018482:Muscle, Skeletal / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism* / Q000503:physiopathology; D019906:Nuclear Magnetic Resonance, Biomolecular; D009711:Nucleotides / Q000378:metabolism; D014020:Tissue Extracts
chemical_list: D000596:Amino Acids; D009711:Nucleotides; D014020:Tissue Extracts; D019344:Lactic Acid; D005947:Glucose