Skeletal muscle metabolism in Duchenne muscular dystrophy (DMD): an in-vitro proton NMR spectroscopy study
| Autor: | Naranamangalam R. Jagannathan, Meher C. Sharma, Chitra Sarkar, Surinder Atri, Uma Sharma |
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| Rok vydání: | 2003 |
| Předmět: |
Muscle tissue
Adult Male medicine.medical_specialty Magnetic Resonance Spectroscopy Adolescent Duchenne muscular dystrophy Biomedical Engineering Biophysics In Vitro Techniques Creatine chemistry.chemical_compound Internal medicine Carnitine medicine Choline Humans Radiology Nuclear Medicine and imaging Muscular dystrophy Isoleucine Child Muscle Skeletal Skeletal muscle Valine Metabolism medicine.disease Immunohistochemistry Muscular Dystrophy Duchenne Endocrinology medicine.anatomical_structure chemistry Child Preschool medicine.drug |
| Zdroj: | Magnetic resonance imaging. 21(2) |
| ISSN: | 0730-725X |
| Popis: | The metabolic differences in the skeletal muscle of patients with Duchenne muscular dystrophy (DMD) and normal subjects (controls) were investigated using in-vitro high-resolution proton NMR spectroscopy. In all, 56 metabolites were unambiguously identified in the perchloric acid extract of muscle tissue using one- and two-dimensional NMR. The concentrations of glycolytic substrate glucose (Glc; p < 0.05), gluconeogenic amino acids such as glutamine (Gln; p < 0.05) and alanine (Ala; p < 0.05) and the glycolytic product lactate (Lac; p < 0.05) were statistically significantly lower in DMD patients as compared to controls. A significant reduction in the concentrations of total creatine (TCr; p < 0.05), glycerophosphoryl choline + phosphoryl choline + carnitine (GPC/PC/Car; p < 0.05), choline (Cho; p < 0.05) and acetate (Ace; p < 0.05) was also observed in these patients. Decrease in the level of glucose may be attributed to the reduction in the concentrations of gluconeogenic substrates or membrane abnormalities in degenerated muscle of DMD patients. Lower levels of choline containing compounds indicate membrane abnormalities. Decrease in the concentration of lactate in the muscle of DMD patients may be due to the reduction in anaerobic glycolytic activity or lower substrate concentration. The decrease in the concentration of acetate may reflect reduced transport of fatty acids into mitochondria due to decreased concentration of carnitine in DMD patients. Kreb's cycle intermediate alpha-ketoglutarate was observed only in the diseased muscle, which is suggestive of predominant oxidative metabolism for energy generation. |
| Databáze: | OpenAIRE |
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