Journal
TRENDS IN MOLECULAR MEDICINE
Volume 12, Issue 6, Pages 279-286Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.molmed.2006.04.003
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Funding
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL064641] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [R01AR049496] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF ENVIRONMENTAL HEALTH SCIENCES [U19ES011384] Funding Source: NIH RePORTER
- NHLBI NIH HHS [HL64641] Funding Source: Medline
- NIAMS NIH HHS [AR49496] Funding Source: Medline
- NIEHS NIH HHS [ES11384] Funding Source: Medline
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Although genetic mutations that are responsible for most of the inherited neuromuscular diseases have been identified, the molecular and cellular mechanisms that cause muscle and nerve depletion are not well understood and therapies are lacking. Histological studies of many neuromuscular diseases indicated that loss of motor-nerve and/or skeletal-muscle function might be due to excessive cell death by apoptosis. Recent studies have confirmed this possibility by showing that pathology in mouse models of amyotrophic lateral sclerosis, congenital muscular dystrophy, oculopharyngeal muscular dystrophy and collagen-VI deficiency, but not Duchenne muscular dystrophy, is significantly ameliorated by genetic or pharmacological interventions that have been designed to inhibit apoptosis. Thus, apoptosis greatly contributes to pathology in mouse models of several neuromuscular diseases, and appropriate antiapoptosis therapy might therefore be beneficial for the corresponding human diseases.
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