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Branched Chain Amino Acids in Inherited Muscle Disease: The Case of Duchenne Muscular Dystrophy

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Branched Chain Amino Acids in Clinical Nutrition

Part of the book series: Nutrition and Health ((NH))

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder affecting boys. The disease is characterized by progressive loss of muscle mass and gain of body fat [1]. Steroids (e.g., prednisone) to ameliorate the symptoms of the disease are the only treatment available at this time, but they exacerbate accumulation of body fat, among other unwanted side effects. Given the dramatic changes in body composition, nutritional strategies are attractive alternatives to improve quality of life in DMD patients, but surprisingly, there have been few systematic studies conducted. Branched chain amino acids, especially leucine, induce protein synthesis, inhibit protein degradation, and induce few to no side effects. Consequently, supplementation of DMD patients with BCAAs is a potential and logical approach to reduce the rate of disease progression. Increased muscle mass could increase strength and mobility of these patients. The current chapter describes the potential benefits of BCAA supplementation for DMD patients.

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Authors and Affiliations

  1. Department of Human Nutrition, Food and Exercise, Virginia Tech, and Institute of Biochemistry and Biophysics, University of Tehran, Tehran, Iran

    Jamshid Davoodi Ph.D.

  2. Department of Human Nutrition, Food and Exercise, Virginia Tech, 338 Wallace Hall, Blacksburg, VA, 24061, USA

    Susan M. Hutson Ph.D.

  3. Department of Human Nutrition, Food and Exercise, Virginia Tech, 1981 Kraft Dr., Blacksburg, VA, 24061, USA

    Robert W. Grange Ph.D.

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  1. Jamshid Davoodi Ph.D.
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  2. Susan M. Hutson Ph.D.
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  3. Robert W. Grange Ph.D.
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Correspondence to Jamshid Davoodi Ph.D. .

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Editors and Affiliations

  1. Intensive Care, Barnet and Chase Farm Hospitals, Royal Free London NHS Foundation Trust, London, United Kingdom

    Rajkumar Rajendram

  2. Diabetes and Nutritional Sciences, Kings College London, London, United Kingdom

    Victor R. Preedy

  3. Department of Biomedical Sciences, University of Westminster, London, United Kingdom

    Vinood B. Patel

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Davoodi, J., Hutson, S.M., Grange, R.W. (2015). Branched Chain Amino Acids in Inherited Muscle Disease: The Case of Duchenne Muscular Dystrophy. In: Rajendram, R., Preedy, V., Patel, V. (eds) Branched Chain Amino Acids in Clinical Nutrition. Nutrition and Health. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-1914-7_22

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  • DOI: https://doi.org/10.1007/978-1-4939-1914-7_22

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