본문바로가기
메뉴바로가기

Global riactice Leader's in Brain Science

정보마당

  • 주요학과행사
  • 공지사항
  • 세미나공지
  • 갤러리
  • 뉴스
  • 기고문
  • 자료실
  • FAQ
  • Q&A

Home > 정보마당 > 세미나공지

세미나공지

10/11 (Thu) 16:00 Special Seminar
이름 : 관리자 | 작성일 : 2018.10.10 10:00 | 조회수 : 563

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
첨부파일
%s1 / %s2
 
List Gallery Webzine RSS FEED

번호 파일 제목 이름 작성일 조회수
png 관리자 2019.02.19 13
291 pptx 관리자 2019.02.15 14
290 pdf 관리자 2019.02.08 24
289 pdf 관리자 2019.01.25 80
288 pdf 관리자 2019.01.24 80
287 pdf 관리자 2019.01.24 71
286 pdf 관리자 2019.01.24 72
285 pdf 관리자 2018.12.06 186
284 pdf 관리자 2018.11.20 242
283 pdf 관리자 2018.11.15 202
282 pdf 관리자 2018.11.15 219
281 pdf 관리자 2018.10.15 431
280 pdf 관리자 2018.10.11 586
279 pdf 관리자 2018.10.11 444
pdf 관리자 2018.10.10 564
277 pdf 관리자 2018.10.02 479
276 pdf 관리자 2018.09.10 706
275 pdf 관리자 2018.09.10 612
274 pdf 관리자 2018.08.29 897
273 jpg 관리자 2018.08.20 7,219
272 pdf 관리자 2018.07.26 7,271