계명대학교 의학도서관 Repository

Bone Marrow-Derived Mesenchymal Stem Cells Prevent the Loss of Niemann-Pick Type C Mouse Purkinje Neurons by Correcting Sphingolipid Metabolism and Increasing Sphingosine-1-phosphate

Metadata Downloads
Author(s)
HYUN LEEJONG KIL LEEWOO-KIE MINJAE-HOON BAEXINGXUAN HEEDWARD H. SCHUCHMANJAE-SUNG BAEHEE KYUNG JIN
Keimyung Author(s)
Bae, Jae Hoon
Department
Dept. of Physiology (생리학)
Journal Title
Stem Cells
Issued Date
2010
Volume
28
Issue
4
Keyword
Niemann-Pick type C disease modelBone marrow-derived mesenchymal stem cellsPurkinje neuronSphingosine-1-phosphateApoptosisTherapeutic potential
Abstract
Niemann-Pick type C (NP-C) disease exhibits neuronal sphingolipid storage and cerebellar Purkinje neuron (PN) loss. Although it is clear that PNs are compromised in this disorder, it remains to be defined how neuronal lipid storage causes the PN loss. Our previous studies have shown that bone marrow-derived mesenchymal stem cells (BM-MSCs) transplantation prevent PN loss in NP-C mice. The aim of the present study was therefore to examine the neuroprotective mechanism of BM-MSCs on PNs. We found that NP-C PNs exhibit abnormal sphingolipid metabolism and defective lysosomal calcium store compared to wild-type mice PNs. BM-MSCs promote the survival of NP-C PNs by correction of the altered calcium homeostasis, restoration of the sphingolipid imbalance, as evidenced by increased sphingosine-1-phosphate levels and decreased sphingosine, and ultimately, inhibition of apoptosis pathways. These effects suggest that BM-MSCs modulate sphingolipid metabolism of endogenous NP-C PNs, resulting in their survival and improved clinical outcome in mice.
Keimyung Author(s)(Kor)
배재훈
Publisher
School of Medicine
Citation
HYUN LEE et al. (2010). Bone Marrow-Derived Mesenchymal Stem Cells Prevent the Loss of Niemann-Pick Type C Mouse Purkinje Neurons by Correcting Sphingolipid Metabolism and Increasing Sphingosine-1-phosphate. Stem Cells, 28(4), 821–831. doi: 10.1002/stem.401
Type
Article
ISSN
1066-5099
DOI
10.1002/stem.401
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/34414
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Physiology (생리학)
공개 및 라이선스
  • 공개 구분공개
파일 목록

Items in Repository are protected by copyright, with all rights reserved, unless otherwise indicated.