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Access through your institution Buy or subscribe Duchenne MD is an X-linked muscle disease caused by an absence of the protein dystrophin. Dystrophin and a similar protein, utrophin, are
structural proteins in skeletal and cardiac muscle cells. Affected boys show signs of disease early in life, stop walking at the beginning of the second decade, and usually die by 20 years
of age. Mutations in genes encoding members of the dystrophin-associated glycoprotein complex lead to different MDs. There is no cure for these diseases, but corticosteroids, including
prednisone and a related compound, deflazacort, can delay the loss of muscle strength and function in Duchenne MD patients. Experimental approaches include gene therapy, cell transplantation
and upregulation of alternative therapeutic proteins. Myostatin is a negative regulator of muscle mass — a mutation in the myostatin gene is responsible for the enormous musculature in
'double-muscled' Belgian blue cattle. After injecting month-old _mdx_ mice with myostatin-blocking antibodies for three months, the authors found that the levels of utrophin in the
muscle were not elevated, suggesting that blockade ameliorated the dystrophic phenoptype _via_ a utrophin-independent mechanism. Elevated serum creatine kinase (CK) concentration is a sign
of dystrophin deficiency in _mdx_ mice; however, after three months of myostatin blockade the serum CK concentration was almost normal. Not all dystrophic changes in _mdx_ mice were reversed
by blocking myostatin; the mice were still susceptible to muscle-fibre damage by lengthening contractions. However, the authors suggest that this might not be the case if the treatment was
started at birth, rather than a month later. This is a preview of subscription content, access via your institution ACCESS OPTIONS Access through your institution Subscribe to this journal
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REFERENCES ORIGINAL RESEARCH PAPER * Bogdanovich, S. et al. Functional improvement of dystrophic muscle by myostatin blockade. _Nature_ 420, 418–421 (2002). Article CAS Google Scholar
FURTHER READING * Andersson, L. Genetic dissection of phenotypic diversity in farm animals. _Nature Rev. Gen._ 2, 130–138 (2001) Article CAS Google Scholar * Lee, S. J. & McPherron,
A. C. et al. Regulation of myostatin activity and muscle growth. _Proc. Natl Acad. Sci. USA_ 98, 9306–9311 (2001). Article CAS Google Scholar Download references Authors * Melanie Brazil
View author publications You can also search for this author inPubMed Google Scholar RELATED LINKS RELATED LINKS WEB SITES Khurana's lab RIGHTS AND PERMISSIONS Reprints and permissions
ABOUT THIS ARTICLE CITE THIS ARTICLE Brazil, M. Body building best without myostatin. _Nat Rev Drug Discov_ 2, 6 (2003). https://doi.org/10.1038/nrd995 Download citation * Issue Date: 01
January 2003 * DOI: https://doi.org/10.1038/nrd995 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content: Get shareable link Sorry, a shareable link
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