کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5534935 1551359 2017 10 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Fibrosis development in early-onset muscular dystrophies: Mechanisms and translational implications
ترجمه فارسی عنوان
توسعه فیبر در دیستروفی عضلانی زودرس: مکانیسم و ​​مفاهیم ترجمه
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی بیولوژی سلول
چکیده انگلیسی

Duchenne muscular dystrophy (DMD) is one of the most devastating neuromuscular genetic diseases caused by the absence of dystrophin. The continuous episodes of muscle degeneration and regeneration in dystrophic muscle are accompanied by chronic inflammation and fibrosis deposition, which exacerbate disease progression. Thus, in addition of investigating strategies to cure the primary defect by gene/cell therapeutic strategies, increasing efforts are being placed on identifying the causes of the substitution of muscle by non-functional fibrotic tissue in DMD, aiming to attenuate its severity. Congenital muscular dystrophies (CMDs) are early-onset diseases in which muscle fibrosis is also present. Here we review the emerging findings on the mechanisms that underlie fibrogenesis in muscular dystrophies, and potential anti-fibrotic treatments.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Seminars in Cell & Developmental Biology - Volume 64, April 2017, Pages 181-190
نویسندگان
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