Abstract:
:It has been suggested that alpha-dystroglycan links the dystrophin-associated protein complex and extracellular matrix and that the absence of dystrophin and alpha-dystroglycan in Duchenne muscular dystrophy (DMD) may lead to the breakdown of this linkage. In the present study, myotubes from DMD patients and murine X-linked muscular dystrophic mice (mdx) were used to measure their adhesive force to the physiological laminin-alpha2 substrate, and it was found that the dystrophic myotubes were selectively unable to sustain adhesion. However, normal and dystrophic myotubes attached equally well to the laminin-alpha1 substrate. As far as we know, this is the first experimental evidence that the absence of dystrophin causes the complete loss of a still unknown laminin-alpha2-dependent adhesion force, therefore suggesting that the primary consequence of Duchenne dystrophy consists of the loss of an authentic mechanical linkage at the level of the alpha-dystroglycan/basal lamina interface.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Angoli D,Corona P,Baresi R,Mora M,Wanke Edoi
10.1016/s0014-5793(97)00460-2subject
Has Abstractpub_date
1997-05-26 00:00:00pages
341-4issue
3eissn
0014-5793issn
1873-3468pii
S0014-5793(97)00460-2journal_volume
408pub_type
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