کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10956809 | 1099457 | 2005 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency
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موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
بیولوژی سلول
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چکیده انگلیسی
NG2, the rat homologue of the human melanoma chondroitin sulfate proteoglycan (MCSP), is a ligand for collagen VI (COL6). We have examined skeletal muscles of patients affected by Ullrich scleroatonic muscular dystrophy (UCMD), an inherited syndrome caused by COL6 genes mutations. A significant decrease of NG2 immunolabeling was found in UCMD myofibers, as well as in skeletal muscle and cornea of COL6 null-mice. In UCMD muscles, truncated NG2 core protein isoforms were detected. However, real-time RT-PCR analysis revealed marked increase in NG2 mRNA content in UCMD muscle compared to controls. We hypothesize that NG2 immunohistochemical and biochemical behavior may be compromised owing to the absence of its physiological ligand. MCSP/NG2 proteoglycan may be considered an important receptor mediating COL6-sarcolemma interactions, a relationship that is disrupted by the pathogenesis of UCMD muscle.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular and Cellular Neuroscience - Volume 30, Issue 3, November 2005, Pages 408-417
Journal: Molecular and Cellular Neuroscience - Volume 30, Issue 3, November 2005, Pages 408-417
نویسندگان
Stefania Petrini, Alessandra Tessa, William B. Stallcup, Patrizia Sabatelli, Mario Pescatori, Betti Giusti, Rosalba Carrozzo, Margherita Verardo, Natascha Bergamin, Marta Columbaro, Camilla Bernardini, Luciano Merlini, Guglielmina Pepe, Paolo Bonaldo,