کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2133177 1086744 2006 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Hypertrophic response of Duchenne and limb-girdle muscular dystrophies is associated with activation of Akt pathway
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی تحقیقات سرطان
پیش نمایش صفحه اول مقاله
Hypertrophic response of Duchenne and limb-girdle muscular dystrophies is associated with activation of Akt pathway
چکیده انگلیسی

Dystrophic muscle undergoes repeated cycles of degeneration/regeneration, characterized by the presence of hypertrophic fibers. In order to elucidate the signaling pathways that govern these events, we investigated Akt activation in normal and dystrophic muscle. Akt is activated in neonatal muscle and in actively dividing myoblasts, supporting a developmental role for Akt signaling. Akt activation was detected at very early, prenecrotic stages of disease pathogenesis, and maximal activation was observed during peak stages of muscle hypertrophy. Duchenne muscular dystrophy patients exhibit a similar pattern of Akt activation. Mice with sarcoglycan-deficient muscular dystrophy possess more severe muscle pathology and display elevated Akt signaling. However, the highest levels of Akt activation were found in dystrophin–utrophin-deficient muscle with very advanced dystrophy. We propose that Akt may serve as an early biomarker of disease and that Akt activation mediates hypertrophy in muscular dystrophy. Current investigations are focused on introducing constitutively active and dominant-negative Akt into prenecrotic mdx mice to determine how early modification of Akt activity influences disease pathogenesis.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Experimental Cell Research - Volume 312, Issue 13, 1 August 2006, Pages 2580–2591
نویسندگان
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