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Global riactice Leader's in Brain Science

10/11 (Thu) 16:00 Special Seminar
Name : 관리자 | Date : 2018.10.10 10:00 | Views : 989

Title: Quest to Cure Krabbe Disease


Speaker: Daesung Shin, Ph.D., Hunter James Kelly Research Institute, State University of New York (Host : Myungin Baek)


Time: 16:00, October 11 (Thu), 2018
        

Venue: Room 114, Building E4, DGIST


Abstract : Krabbe Disease (KD, also known as ‘globoid cell leukodystrophy’) is a fatal lysosomal storage disorder caused by mutations in galactosylceramidase (GALC), which cause demyelinating neurodegeneration. Unfortunately, there is no cure for KD and we have a limited understanding of KD pathogenesis. Hematopoietic stem cell transplantation (HSCT) partially attenuates the course of KD only if performed before the onset of symptoms, presumably because stem cell derivatives secrete GALC that is uptaken by myelinating glia. However, it is not clear if only myelin-forming glia need to be corrected and at which developmental stage. Furthermore, accumulation of the lipid psychosine due to GALC deficiency contributes to KD by killing myelin-forming glia and neurons, but the relative importance of psychosine, its origin and the sequence of pathogenic events is unclear. We recently developed a conditional Galc floxed mouse and found that: 1) A KD phenotype is delayed (~25 days) when Galc ablation is induced ubiquitously [Galc-iKO] after postnatal day (P) 6, as compared to induction at P4 or before, suggesting that there is a defined vulnerable period in which GALC deficiency initiates KD pathogenesis; 2) Oligodendrocyte (OL)-specific Galc conditional knockout [Galc-CKO] causes tremor, wasting, motor defects, demyelination with substantial psychosine accumulation, but milder pathology than Galc-iKO mice, suggesting that Galc deficiency in OLs alone may not be sufficient to trigger a complete KD phenotype. These findings are important to define the therapeutic window to treat and allow the identification of novel specific target cells for therapy.


Person in charge : Sora Lee


Contact: srlee@dgist.ac.kr, 053)785-6102

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