Development of therapies against neuromuscular diseases causing muscle atrophy

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Nihon Shinkei Seishin Yakurigaku Zasshi. 2006 Nov;26(5-6):229-33.

Development of therapies against neuromuscular diseases causing

muscle atrophy

Tsuchida K.

Division for Therapies Against Intractable Diseases, Institute for

Comprehensive Medical Science (ICMS), Fujita Health University,

Toyoake, 470-1192 Japan.

Skeletal muscles become atrophied by muscular disorders such as

muscular dystrophy, wasting and even aging. In addition to muscle

atrophy, progressive muscle damage, inflammation and replacement of

muscle fibers with fibrous and fatty tissues are observed in

muscular dystrophy. Neuronal innervation is required for skeletal

muscle, and muscles become atrophic when motor neurons are affected

by neurodegenerative disorders such as amyotrophic lateral

sclerosis.

Restoring muscle mass and function lost by diseases such as muscular

dystrophy and neurodegenerative disorders is important. There are

three rational therapies for muscular dystrophy and related

diseases: gene therapy, cell therapy and drug therapy. Gene

therapies to replace the defective genes have been tried with

various degrees of effectiveness.

Multiple myogenic stem cells including satellite cells, bone marrow

cells, muscle side population cells, muscle-derived stem cells and

mesoangioblast have been characterized. Cell therapies using these

stem cells are one of the promising therapies for neuromuscular

diseases causing muscle atrophy.

As pharmacological drug therapies, increasing skeletal muscle mass

by myostatin inhibition is quite promising and will be applied

clinically in the near future.