본문바로가기
메뉴바로가기

Global riactice Leader's in Brain Science

10/11 (Thu) 16:00 Special Seminar
Name : 관리자 | Date : 2018.10.10 10:00 | Views : 737

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

IP : 10.140.1.*** share
QRcode
  • twitter
  • facebook
  • me2day
  • yozm
  • clog
  • delicious
Attachments
%s1 / %s2
 
List Gallery Webzine RSS FEED

No File Subject Name Date Views
png 관리자 2019.02.19 1,023
300 pdf 관리자 2019.04.16 6
299 pdf 관리자 2019.04.15 5
298 png 관리자 2019.04.11 12
297 pdf 관리자 2019.04.05 71
296 pdf 관리자 2019.03.29 93
295 pdf 관리자 2019.03.22 96
294 pdf 관리자 2019.03.21 75
293 pdf 관리자 2019.03.06 113
292 pptx 관리자 2019.02.28 91
291 pptx 관리자 2019.02.15 135
290 pdf 관리자 2019.02.08 136
289 pdf 관리자 2019.01.25 195
288 pdf 관리자 2019.01.24 206
287 pdf 관리자 2019.01.24 194
286 pdf 관리자 2019.01.24 199
285 pdf 관리자 2018.12.06 309
284 pdf 관리자 2018.11.20 370
283 pdf 관리자 2018.11.15 325
282 pdf 관리자 2018.11.15 345
281 pdf 관리자 2018.10.15 564