NDLI: What do mouse models of muscular dystrophy tell us about the DAPC and its components?

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What do mouse models of muscular dystrophy tell us about the DAPC and its components?

Content Provider	Scilit
Author	Whitmore, Charlotte Morgan, Jennifer
Copyright Year	2014
Description	Journal: International journal of experimental pathology There are over 30 mouse models with mutations or inactivations in the dystrophin-associated protein complex. This complex is thought to play a crucial role in the functioning of muscle, as both a shock absorber and signalling centre, although its role in the pathogenesis of muscular dystrophy is not fully understood. The first mouse model of muscular dystrophy to be identified with a mutation in a component of the dystrophin-associated complex (dystrophin) was the mdx mouse in 1984. Here, we evaluate the key characteristics of the mdx in comparison with other mouse mutants with inactivations in DAPC components, along with key modifiers of the disease phenotype. By discussing the differences between the individual phenotypes, we show that the functioning of the DAPC and consequently its role in the pathogenesis is more complicated than perhaps currently appreciated.
Related Links	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4285463/pdf
Ending Page	377
Page Count	13
Starting Page	365
ISSN	09599673
e-ISSN	13652613
DOI	10.1111/iep.12095
Journal	International journal of experimental pathology
Issue Number	6
Volume Number	95
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2014-09-30
Access Restriction	Open
Subject Keyword	Journal: International journal of experimental pathology Muscular Dystrophy
Content Type	Text
Resource Type	Article
Subject	Cell Biology Molecular Biology Pathology and Forensic Medicine

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