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Aging: Influence on Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD)

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Models, Molecules and Mechanisms in Biogerontology

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Abstract

Muscular dystrophies are genetic diseases of muscles. These diseases are characterized by progressive muscle wasting and weakness of variable distribution and severity. Selective involvement, significant wasting, and weakness of muscles are significant characteristics of muscular dystrophies. The most important types of X-linked dystrophy are Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). DMD is the most rapidly progressive and lethal form of dystrophy, but BMD is a milder form. Both the types of dystrophy are caused by a mutation in a specific gene (dystrophin gene) within the X chromosome (gene map locus 12q21, Xp21.2) that affords commands for the formation of the dystrophin protein, and this protein is an important structural component of muscle cell. Aging is an important parameter for patients with DMD and BMD. This influences the clinical symptoms, disease progression, serum enzyme abnormalities, morphological changes in muscle, and medications for DMD and BMD patients. Aging factor is responsible for pathogenesis in DMD and BMD. With increased knowledge about the understanding of DMD and BMD, still there is an uncertainty of the exact mechanism of progressive muscle degeneration in these disorders. On the basis of numerous reports, oxidative stress may be one of the causes for the muscle degeneration in DMD and BMD, which increases with age. The present chapter described the effect of aging on all the parameters of disease in DMD and BMD.

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Abbreviations

BMD:

Becker muscular dystrophy

CK:

creatine kinase

CMD:

congenital muscular dystrophy

DM:

myotonic dystrophy

DMD:

Duchenne muscular dystrophy

EDMD:

Emery-Dreifus muscular dystrophy

EMG:

electromyography

FSHD:

facioscapulohumeral muscular dystrophy

LGMD-2B:

limb-girdle muscular dystrophy-2B

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Acknowledgments

Financial support from D.S. Kothari postdoctoral fellowship, University Grant Commission (UGC) Government of India, New Delhi, is gratefully acknowledged.

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Authors and Affiliations

  1. School of Life Sciences, Jawaharlal Nehru University, New Delhi, India

    Niraj Kumar Srivastava & Deepak Sharma

  2. Department of Neurology, UP University of Medical Sciences, Saifai, India

    Ramakant Yadav

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Correspondence to Deepak Sharma .

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  1. School of Life Sciences, Jawaharlal Nehru University, New Delhi, India

    Pramod C. Rath

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Srivastava, N.K., Yadav, R., Sharma, D. (2019). Aging: Influence on Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD). In: Rath, P. (eds) Models, Molecules and Mechanisms in Biogerontology. Springer, Singapore. https://doi.org/10.1007/978-981-13-3585-3_8

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