Disease-proportional proteasomal degradation of missense dystrophins
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چکیده
منابع مشابه
Disease-proportional proteasomal degradation of missense dystrophins.
The 427-kDa protein dystrophin is expressed in striated muscle where it physically links the interior of muscle fibers to the extracellular matrix. A range of mutations in the DMD gene encoding dystrophin lead to a severe muscular dystrophy known as Duchenne (DMD) or a typically milder form known as Becker (BMD). Patients with nonsense mutations in dystrophin are specifically targeted by stop c...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2015
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1508755112