Document Detail

Immortalization of murine muscle cells from lysosomal alpha-glucosidase deficient mice: a new tool to study pathophysiology and assess therapeutic strategies for Pompe disease.
MedLine Citation:
PMID:  19665008     Owner:  NLM     Status:  MEDLINE    
Glycogen storage disease type II (GSDII) is an autosomal recessive disorder caused by defects in the acid alpha-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.
Gaëlle Douillard-Guilloux; Vincent Mouly; Catherine Caillaud; Emmanuel Richard
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-08-06
Journal Detail:
Title:  Biochemical and biophysical research communications     Volume:  388     ISSN:  1090-2104     ISO Abbreviation:  Biochem. Biophys. Res. Commun.     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-08-31     Completed Date:  2009-09-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372516     Medline TA:  Biochem Biophys Res Commun     Country:  United States    
Other Details:
Languages:  eng     Pagination:  333-8     Citation Subset:  IM    
Institut Cochin, Université Paris Descartes. CNRS (UMR 8104), Paris, France; INSERM, U567, Paris, France.
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MeSH Terms
Cell Line*
Disease Models, Animal
Glycogen Storage Disease Type II / drug therapy,  enzymology*
Lysosomes / enzymology
Mice, Knockout
Muscle Development
Myoblasts / cytology*,  enzymology,  physiology
alpha-Glucosidases / genetics*
Reg. No./Substance:

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