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Publication : The cysteine-rich and C-terminal domains of dystrophin are not required for normal costameric localization in the mouse.

First Author  Maconochie MK Year  1996
Journal  Transgenic Res Volume  5
Issue  2 Pages  123-30
PubMed ID  8866894 Mgi Jnum  J:72373
Mgi Id  MGI:2152548 Doi  10.1007/BF01969430
Citation  Maconochie MK, et al. (1996) The cysteine-rich and C-terminal domains of dystrophin are not required for normal costameric localization in the mouse. Transgenic Res 5(2):123-30
abstractText  Dystrophin has a modular structure and is believed to be critical for muscle cell cytoarchitecture by linking the cytoskeleton to the extracellular matrix. The N-terminus binds to actin and two domains at the C-terminus, the cysteine-rich and C-terminal domains, are associated with the sarcolemma indirectly via the dystroglycan complex. We have generated a mutation in mouse embryonic stem (ES) cells which serves to delete the cysteine-rich and C-terminal domains to address directly their role. We show that these two domains are not necessary for normal costameric organization at the sarcolemma in myotubes derived from the mutant cell line. Furthermore sarcolemmal localization is also apparent in mouse chimaeric muscle in vivo.
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