Abnormal lipid metabolism in skeletal muscle tissue of patients with muscular dystrophy: In vitro,high-resolution NMR spectroscopy based observation in early phase of the disease |
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| Institution: | 1. Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India;2. Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India;3. Center of Biomedical Research, SGPGIMS Campus, Raebareli Road, Lucknow 226014, India;1. Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA, USA;2. UC Berkeley-UCSF Graduate Program in Bioengineering, University of California, San Francisco and University of California, Berkeley, CA, USA;3. HeartVista Inc., Los Altos, CA, USA;4. Department of Cell and Tissue Biology, University of California, San Francisco, CA, USA;1. Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Thammasat University, Pathumthani 12120, Thailand;2. Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10600, Thailand;1. Center for Clinical Genetics, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China;2. Department of Genetics, Shanghai Institute of Pediatric Research, Shanghai, China;3. Department of Pediatric Neurology, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China |
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| Abstract: | PurposeQualitative (assignment of lipid components) and quantitative (quantification of lipid components) analysis of lipid components were performed in skeletal muscle tissue of patients with muscular dystrophy in early phase of the disease as compared to control/normal subjects.MethodsProton nuclear magnetic resonance (NMR) spectroscopy based experiment was performed on the lipid extract of skeletal muscle tissue of patients with muscular dystrophy in early phase of the disease and normal individuals for the analysis of lipid components triglycerides, phospholipids, total cholesterol and unsaturated fatty acids (arachidonic, linolenic and linoleic acid)]. Specimens of muscle tissue were obtained from patients with Duchenne muscular dystrophy (DMD) n = 11; Age, Mean ± SD; 9.2 ± 1.4 years; all were males], Becker muscular dystrophy (BMD) n = 12; Age, Mean ± SD; 21.4 ± 5.0 years; all were males], facioscapulohumeral muscular dystrophy (FSHD) n = 11; Age, Mean ± SD; 23.7 ± 7.5 years; all were males] and limb girdle muscular dystrophy-2B (LGMD-2B) n = 18; Age, Mean ± SD; 24.2 ± 4.1 years; all were males]. Muscle specimens were also obtained from n = 30; Mean age ± SD 23.1 ± 6.0 years; all were males] normal/control subjects.ResultsAssigned lipid components in skeletal muscle tissue were triglycerides (TG), phospholipids (PL), total cholesterol (CHOL) and unsaturated fatty acids (arachidonic, linolenic and linoleic acid)]. Quantity of lipid components was observed in skeletal muscle tissue of DMD, BMD, FSHD and LGMD-2B patients as compared to control/normal subjects. TG was significantly elevated in muscle tissue of DMD, BMD and LGMD-2B patients. Increase level of CHOL was found only in muscle of DMD patients. Level of PL was found insignificant for DMD, BMD and LGMD-2B patients. Quantity of TG, PL and CHOL was unaltered in the muscle of patients with FSHD as compared to control/normal subjects. Linoleic acids were significantly reduced in muscle tissue of DMD, BMD, FSHD and LGMD-2B as compared to normal/control individuals.ConclusionsResults clearly indicate alteration of lipid metabolism in patients with muscular dystrophy in early phase of the disease. Moreover, further evaluation is required to understand whether these changes are primary or secondary to muscular dystrophy. In future, these findings may prove an additional and improved approach for the diagnosis of different forms of muscular dystrophy. |
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