کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8480185 | 1551373 | 2016 | 25 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Connexins in skeletal muscle development and disease
دانلود مقاله + سفارش ترجمه
دانلود مقاله ISI انگلیسی
رایگان برای ایرانیان
کلمات کلیدی
Myogenesismyogenic regulatory factor 4ODDDMrf4Oculodentodigital dysplasiaMyf5Connexin45Pax7TRPC1Connexin40Connexin43GJICCX45CX40CX43Adenosine Triphosphate - آدنوزین تری فسفاتATP - آدنوزین تری فسفات یا ATPgap junctions - اتصالات شکاف دارgap junctional intercellular communication - ارتباط بین سلولی بین دو سلولیpaired box 7 - جعبه زوج 7Myogenic Factor 5 - عامل فاکتور 5Skeletal muscle - عضله اسکلتیconnexin - کنسکسین
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
بیولوژی سلول
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Gap junctions consist of clusters of intercellular channels composed of connexins that connect adjacent cells and allow the exchange of small molecules. While the 21 member multi-gene family of connexins are ubiquitously found in humans, only Cx39, Cx40, Cx43 and Cx45 have been documented in developing myoblasts and injured adult skeletal muscle while healthy adult skeletal muscle is devoid of connexins. The use of gap junctional blockers and cultured myoblast cell lines have suggested that these connexins play a critical role in myotube formation and muscle regeneration. More recent genetically-modified mouse models where Cx43 function is greatly compromized or ablated have further supported a role for Cx43 in regulating skeletal muscle development. In the last decade, we have become aware of a cohort of patients that have a development disorder known as oculodentodigital dysplasia (ODDD). These patients harbor either gain or loss of Cx43 function gene mutations that result in many organ anomalies raising questions as to whether they suffer from defects in skeletal muscle formation or regeneration upon injury. Interesting, some ODDD patients report muscle weakness and loss of limb control but it is not clear if this is neurogenic or myogenic in origin. This review will focus on the role connexins play in muscle development and repair and discuss the impact of Cx43 mutants on muscle function.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Seminars in Cell & Developmental Biology - Volume 50, February 2016, Pages 67-73
Journal: Seminars in Cell & Developmental Biology - Volume 50, February 2016, Pages 67-73
نویسندگان
Peter A. Merrifield, Dale W. Laird,