Title |
Characterization of adipose-derived stromal/stem cells from the twitcher mouse model of krabbe disease
|
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Published in |
BMC Molecular and Cell Biology, April 2013
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DOI | 10.1186/1471-2121-14-20 |
Pubmed ID | |
Authors |
Xiujuan Zhang, Julie A Semon, Shijia Zhang, Amy L Strong, Brittni A Scruggs, Jeffrey M Gimble, Bruce A Bunnell |
Abstract |
Krabbe disease, also known as globoid cell leukodystrophy, is an autosomal recessive neurodegenerative disease caused by the genetic deficiency of galactocerebrosidase (GALC), a lysosomal enzyme responsible for the degradation of several glycosphingolipids like psychosine and galactosylceramide. In order to investigate whether GALC deficiency in Krabbe disease affects adipose-derived stromal/stem cell (ASC) properties and if the ASCs could be used as a source of autologous stem cell therapy for patients with Krabbe disease, ASCs isolated from subcutaneous adipose tissue of Twitcher mice (a murine model of Krabbe disease) and their normal wild type littermates were cultured, expanded, and characterized for their cell morphology, surface antigen expression, osteogenic and adipogenic differentiation, colony forming units, growth kinetics, and immune regulatory capacities in vitro. |
X Demographics
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Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
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Italy | 2 | 8% |
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Researcher | 6 | 25% |
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Professor | 2 | 8% |
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