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The role of apoptosis in neuromuscular diseases and prospects for anti-apoptosis therapy
- Source :
- Trends in Molecular Medicine. 12:279-286
- Publication Year :
- 2006
- Publisher :
- Elsevier BV, 2006.
-
Abstract
- 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 anti-apoptosis therapy might therefore be beneficial for the corresponding human diseases.
- Subjects :
- Pathology
medicine.medical_specialty
Programmed cell death
Duchenne muscular dystrophy
Apoptosis
Minocycline
Biology
medicine.disease_cause
Bioinformatics
Poly(A)-Binding Protein II
Muscular Dystrophies
Oculopharyngeal muscular dystrophy
Mice
Superoxide Dismutase-1
Muscular Dystrophy, Oculopharyngeal
medicine
Animals
Humans
Agrin
Amyotrophic lateral sclerosis
Muscular dystrophy
Molecular Biology
Clinical Trials as Topic
Mutation
Superoxide Dismutase
Amyotrophic Lateral Sclerosis
Genetic Therapy
medicine.disease
Anti-Bacterial Agents
Disease Models, Animal
Gene Expression Regulation
Doxycycline
Congenital muscular dystrophy
Molecular Medicine
Laminin
Apoptosis Regulatory Proteins
Subjects
Details
- ISSN :
- 14714914
- Volume :
- 12
- Database :
- OpenAIRE
- Journal :
- Trends in Molecular Medicine
- Accession number :
- edsair.doi.dedup.....bcf7380c93f6114cfb397ff4189dca90