Genetic Modifiers for Neuromuscular Diseases

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Genetic Modifiers for Neuromuscular Diseases.

Neuromuscular diseases, which encompass disorders that affect muscle and its innervation, are highly heritable. Genetic diagnosis now frequently pinpoints the primary mutation responsible for a given neuromuscular disease. However, the results from genetic testing indicate that neuromuscular disease phenotypes may vary widely, even in individuals with the same primary disease-causing mutation. ...

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Genetic modifiers of ambulation in the cooperative international Neuromuscular research group Duchenne natural history study

OBJECTIVE We studied the effects of LTBP4 and SPP1 polymorphisms on age at loss of ambulation (LoA) in a multiethnic Duchenne muscular dystrophy (DMD) cohort. METHODS We genotyped SPP1 rs28357094 and LTBP4 haplotype in 283 of 340 participants in the Cooperative International Neuromuscular Research Group Duchenne Natural History Study (CINRG-DNHS). Median ages at LoA were compared by Kaplan-Me...

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Genetic Modifiers for the Long-QT Syndrome

The long-QT syndrome (LQTS), probably the best-known genetic disorder causing life-threatening arrhythmias, has become a useful paradigm to study sudden cardiac death in the young. An intriguing feature of LQTS is its incomplete penetrance and variable expressivity which are commonly observed even among members of the same family, all carriers of the same mutation. There is a consensus that thi...

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Gene and splicing therapies for neuromuscular diseases.

Neuromuscular disorders (NMD) are heterogeneous group of genetic diseases characterized by muscle weakness and wasting. Duchenne Muscular dystrophy (DMD) and Spinal muscular atrophy (SMA) are two of the most common and severe forms in humans and although the molecular mechanisms of these diseases have been extensively investigated, there is currently no effective treatment. However, new gene-ba...

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Stem cell therapies for neuromuscular diseases.

The promise of stem cells for therapy of a variety of disease conditions is one of the most heavily marketed commodities of modern biotechnology. Interest in this prospect has opened up and destabilized a large area of developmental biology, challenging some of its major tenets, and becoming a source of controversy. Much of the disagreement arises from apparently simple conflicts of findings, w...

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ژورنال

عنوان ژورنال: Journal of Neuromuscular Diseases

سال: 2014

ISSN: 2214-3599

DOI: 10.3233/jnd-140023