Literature DB >> 28069416

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.

Niraj Kumar Srivastava1, Ramakant Yadav2, Somnath Mukherjee2, Lily Pal3, Neeraj Sinha4.   

Abstract

PURPOSE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
Copyright © 2017 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  BMD; Cholesterol; DMD; Essential fatty acids; FSHD; LGMD-2B; Muscular dystrophy; NMR spectroscopy; Phospholipids; Triglycerides

Mesh:

Substances:

Year:  2017        PMID: 28069416     DOI: 10.1016/j.mri.2017.01.001

Source DB:  PubMed          Journal:  Magn Reson Imaging        ISSN: 0730-725X            Impact factor:   2.546


  15 in total

1.  High prevalence of plasma lipid abnormalities in human and canine Duchenne and Becker muscular dystrophies depicts a new type of primary genetic dyslipidemia.

Authors:  Zoe White; Chady H Hakim; Marine Theret; N Nora Yang; Fabio Rossi; Dan Cox; Gordon A Francis; Volker Straub; Kathryn Selby; Constadina Panagiotopoulos; Dongsheng Duan; Pascal Bernatchez
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2.  Increased nonHDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B.

Authors:  Stephanie L Sellers; Nadia Milad; Zoe White; Chris Pascoe; Rayleigh Chan; Geoffrey W Payne; Chun Seow; Fabio Rossi; Michael A Seidman; Pascal Bernatchez
Journal:  J Lipid Res       Date:  2017-11-25       Impact factor: 5.922

3.  Age-dependent changes in metabolite profile and lipid saturation in dystrophic mice.

Authors:  Brittany Lee-McMullen; Stephen M Chrzanowski; Ravneet Vohra; Sean C Forbes; Krista Vandenborne; Arthur S Edison; Glenn A Walter
Journal:  NMR Biomed       Date:  2019-03-08       Impact factor: 4.044

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Journal:  Quant Imaging Med Surg       Date:  2020-07

Review 5.  Non-Glycanated Biglycan and LTBP4: Leveraging the extracellular matrix for Duchenne Muscular Dystrophy therapeutics.

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Review 6.  Proceedings of the Ninth HDL (High-Density Lipoprotein) Workshop: Focus on Cardiovascular Disease.

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Journal:  Arterioscler Thromb Vasc Biol       Date:  2019-10-10       Impact factor: 8.311

7.  β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy.

Authors:  Letizia Brogi; Maria Marchese; Alessandro Cellerino; Rosario Licitra; Valentina Naef; Serena Mero; Carlo Bibbiani; Baldassare Fronte
Journal:  Nutrients       Date:  2021-05-12       Impact factor: 5.717

8.  Increased plasma lipid levels exacerbate muscle pathology in the mdx mouse model of Duchenne muscular dystrophy.

Authors:  Nadia Milad; Zoe White; Arash Y Tehrani; Stephanie Sellers; Fabio M V Rossi; Pascal Bernatchez
Journal:  Skelet Muscle       Date:  2017-09-12       Impact factor: 4.912

9.  Plasma lipidomic analysis shows a disease progression signature in mdx mice.

Authors:  Roula Tsonaka; Alexandre Seyer; Annemieke Aartsma-Rus; Pietro Spitali
Journal:  Sci Rep       Date:  2021-06-21       Impact factor: 4.379

10.  Altered cholesterol homeostasis in critical illness-induced muscle weakness: effect of exogenous 3-hydroxybutyrate.

Authors:  Chloë Goossens; Ruben Weckx; Greet Van den Berghe; Lies Langouche; Sarah Derde; Sarah Vander Perre; Inge Derese; Paul P Van Veldhoven; Bart Ghesquière
Journal:  Crit Care       Date:  2021-07-17       Impact factor: 9.097

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