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Metabolic Disturbance in Patients with Muscular Dystrophy and Reflection of Altered Enzyme Activity in Dystrophic Muscle: One Critical View

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Open Access
Article Type Review Article
Subject Medicine Group
OCLC 8840461174
Niraj Kumar Srivastava*, Somnath Mukherjee and Vijay Nath Mishra
Issue: Volume1-Issue8
Pages: 393-403
Received: 2020-12-04
Accepted: 2020-12-14
Published: 2020-12-15

Abstract

Muscular dystrophies are inherited myogenic diseases and considered by progressive muscle wasting and weakness with variable distribution and severity. The essential characteristics of muscular dystrophies are selective involvement, significant wasting and weakness of muscles. The most common and frequent types of muscular dystrophies are Duchenne Muscular Dystrophy (DMD), Becker Muscular Dystrophy (BMD), Facioscapulohumeral Dystrophy (FSHD) and Limb Girdle Muscular Dystrophy (LGMD). Metabolic disturbance is observed in muscular dystrophy patients (DMD, BMD, FSHD and LGMD-2B). Alteration in the level of metabolites (BCAA, Glu/ Gln, Ace, alanine, glucose, histidine, propionate, tyrosine and fumarate) in dystrophic muscle reflects the alteration in the activity of enzymes. Collectively, these observations propose that there is alteration in the rate of glycolysis, TCA cycle, fatty acid oxidation, gluconeogenesis pathway and protein metabolism (catabolism & anabolism) in the muscular dystrophy patients. Metabolic disturbance, further provide the explanation about the pathophysiology of muscular dystrophy.

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© 2020 Srivastava NK, et al. Distributed under Creative Commons CC-BY 4.0 Creative CommonsAttribution

How to cite this article

Srivastava NK, Mukherjee S, Mishra VN. Metabolic Disturbance in Patients with Muscular Dystrophy and Reflection of Altered Enzyme Activity in Dystrophic Muscle: One Critical View. J Biomed Res Environ Sci. 2020 Dec 15; 1(8): 393-403. doi: 10.37871/jbres1171, Article ID: jbres1171

Subject area(s)

Metabolic SyndromesMetabolism

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