Srivastava Niraj Kumar, Yadav Ramakant, Mukherjee Somnath, Pal Lily, Sinha Neeraj
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India.
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India.
Magn Reson Imaging. 2017 May;38:163-173. doi: 10.1016/j.mri.2017.01.001. Epub 2017 Jan 7.
Qualitative (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.
Proton 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±1.4years; all were males], Becker muscular dystrophy (BMD) [n=12; Age, Mean±SD; 21.4±5.0years; all were males], facioscapulohumeral muscular dystrophy (FSHD) [n=11; Age, Mean±SD; 23.7±7.5years; all were males] and limb girdle muscular dystrophy-2B (LGMD-2B) [n=18; Age, Mean±SD; 24.2±4.1years; all were males]. Muscle specimens were also obtained from [n=30; Mean age±SD 23.1±6.0years; all were males] normal/control subjects.
Assigned 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.
Results 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.
对肌营养不良症早期患者的骨骼肌组织进行脂质成分的定性（脂质成分的分配）和定量（脂质成分的定量）分析，并与对照/正常受试者进行比较。
对肌营养不良症早期患者和正常个体的骨骼肌组织脂质提取物进行基于质子核磁共振（NMR）光谱的实验，以分析脂质成分[甘油三酯、磷脂、总胆固醇和不饱和脂肪酸（花生四烯酸、亚麻酸和亚油酸）]。肌肉组织样本取自杜兴氏肌营养不良症（DMD）患者[n = 11；年龄，平均值±标准差；9.2±1.4岁；均为男性]、贝克氏肌营养不良症（BMD）患者[n = 12；年龄，平均值±标准差；21.4±5.0岁；均为男性]、面肩肱型肌营养不良症（FSHD）患者[n = 11；年龄，平均值±标准差；23.7±7.5岁；均为男性]和肢带型肌营养不良症2B型（LGMD - 2B）患者[n = 18；年龄，平均值±标准差；24.2±4.1岁；均为男性]。还从[n = 30；平均年龄±标准差23.1±6.0岁；均为男性]正常/对照受试者获取肌肉样本。
骨骼肌组织中确定的脂质成分有甘油三酯（TG）、磷脂（PL）、总胆固醇（CHOL）和不饱和脂肪酸（花生四烯酸、亚麻酸和亚油酸）]。与对照/正常受试者相比，观察到DMD、BMD、FSHD和LGMD - 2B患者骨骼肌组织中的脂质成分数量。DMD、BMD和LGMD - 2B患者肌肉组织中的TG显著升高。仅在DMD患者的肌肉中发现CHOL水平升高。发现DMD、BMD和LGMD - 2B患者的PL水平无显著变化。与对照/正常受试者相比，FSHD患者肌肉中的TG、PL和CHOL数量未改变。与正常/对照个体相比，DMD、BMD、FSHD和LGMD - 2B患者肌肉组织中的亚油酸显著减少。
结果清楚地表明肌营养不良症早期患者存在脂质代谢改变。此外，需要进一步评估以了解这些变化是原发性的还是肌营养不良症继发的。未来，这些发现可能为不同形式的肌营养不良症诊断提供一种额外且改进的方法。
