کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1905849 1534744 2007 10 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Dystrophin, its interactions with other proteins, and implications for muscular dystrophy
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
پیش نمایش صفحه اول مقاله
Dystrophin, its interactions with other proteins, and implications for muscular dystrophy
چکیده انگلیسی

Duchenne muscular dystrophy is the most prevalent and severe form of human muscular dystrophy. Investigations into the molecular basis for Duchenne muscular dystrophy were greatly facilitated by seminal studies in the 1980s that identified the defective gene and its major protein product, dystrophin. Biochemical studies revealed its tight association with a multi-subunit complex, the so-named dystrophin–glycoprotein complex. Since its description, the dystrophin–glycoprotein complex has emerged as an important structural unit of muscle and also as a critical nexus for understanding a diverse array of muscular dystrophies arising from defects in several distinct genes. The dystrophin homologue utrophin can compensate at the cell/tissue level for dystrophin deficiency, but functions through distinct molecular mechanisms of protein–protein interaction.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1772, Issue 2, February 2007, Pages 108–117
نویسندگان
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