Article
Characterization of adipose-derived stromal/stem cells from the Twitcher mouse model of Krabbe disease.
BMC cell biology - 16 Apr 2013
Zhang Xiujuan, Semon Julie A, Zhang Shijia, Strong Amy L, Scruggs Brittni A, Gimble Jeffrey M, Bunnell Bruce A
Abstract excerpt
BACKGROUND: 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...
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