Characterization of adipose-derived stromal/stem cells from the Twitcher mouse model of Krabbe disease.

[krabbe disease]

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 .ASCs from Twitcher mice (TwiASCs ), when compared to ASCs from normal mice (WtASCs ), have a reduced osteogenic differentiation potential , have less self-replicating and proliferative capacity , although they have the same fibroblast morphologies and cell sizes . However , surprisingly , the TwiASCs demonstrated similar immune-suppressive capacities as their counterparts WtASCs did when they were transwell co -cultured with macrophages in vitro .This study reveals that Twitcher ASCs exhibit differences in the biologic potential when compared to their counterparts from normal mice . The changes in Twitcher ASCs may be influenced by the GALC deficiency in Twitcher mice . Nevertheless , none of the changes preclude the use of the TwiASCs for autologous applications .

Diseases
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Diseases presenting "similar immune-suppressive capacities" symptom

krabbe disease

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