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Utrophin |
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updated 9/10/02 |
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A
ubiquitously expressed analog of dystrophin which forms a link between the
plasma-membrane and the underlying actin-based cytoskeleton.
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Actin binding
Utrophin binds actin through tandem CH-domains
as do the various other actin-binding proteins in the spectrin
super-family.
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Utrophin a cure for Muscular Dystrophin?
The mouse model for muscular dystrophy, the MDX
mouse, is not very good as this mouse lacks the phenotype of muscle
wasting and degeneration (). This is largely because in mice,
utrophin is also expressed in muscle and this compensates for the lack
of functional dystrophin. Mice lacking both utrophin and
dystrophin exhibit the MD phenotype (). There is good reason to
suppose that utrophin could compensate for dystrophin in humans too as
the muscles of the human eye (the ocular muscles) remain unaffected by
MD, and express utrophin ().
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SteveWinder Lab in Glasgow, Scotland |
CH-domains |
Dystrophin |
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References:-
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actin binding and cellular targeting. J. Cell Sci. 108, 63-71.
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Calcium/calmodulin-dependent regulation of the NH2-terminal
F-actin binding domain of utrophin. FEBS Letters. 357, 125-128. |
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