کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1933046 1050600 2009 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Immortalization of murine muscle cells from lysosomal α-glucosidase deficient mice: A new tool to study pathophysiology and assess therapeutic strategies for Pompe disease
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
Immortalization of murine muscle cells from lysosomal α-glucosidase deficient mice: A new tool to study pathophysiology and assess therapeutic strategies for Pompe disease
چکیده انگلیسی

Glycogen storage disease type II (GSDII) is an autosomal recessive disorder caused by defects in the acid α-glucosidase (GAA) gene leading to lysosomal glycogen accumulation, mainly in cardiac and muscle tissues. In order to facilitate biological investigation on this disease and to avoid time-consuming direct cell isolation and culture, we have established murine myogenic GSDII cell lines. Lentiviral/retroviral expression of SV40 T antigen, Bmi-1 or cyclin-dependent kinase 4 (CDK4) genes was used to induce the immortalization of primary satellite cells from GSDII mice. The resulting immortalized myoblasts exhibit phenotypic characteristics of their parental cells, including profound GAA deficiency, glycogen accumulation and the ability to fully differentiate into myotubes when placed in proper culture conditions. These cell lines will constitute a powerful tool for both basic and applied studies focused on a better understanding of the pathophysiological mechanisms involved in GSDII and for assessing putative therapeutic strategies.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 388, Issue 2, 16 October 2009, Pages 333–338
نویسندگان
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