Mouse neural progenitor cells differentiate into oligodendrocytes in the brain of a knockout mouse model of Canavan disease

Canavan disease (CD) is an autosomal recessive disorder that leads to spongy degeneration in the white matter of the brain. Aspartoacylase (ASPA) synthesizing cells, oligodendrocytes, are lost in CD. Transplantation of neural progenitor cells (NPCs) offers an interesting therapeutic approach for tre...

Full description

Saved in:
Bibliographic Details
Published in:Brain research. Developmental brain research 2004-10, Vol.153 (1), p.19-27
Main Authors: Surendran, Sankar, Shihabuddin, Lamya S., Clarke, Jennifer, Taksir, Tatyana V., Stewart, Gregory R., Parsons, Geoffrey, Yang, Wendy, Tyring, Stephen K., Michals-Matalon, Kimberlee, Matalon, Reuben
Format: Article
Language:English
Subjects:
Amidohydrolases - genetics
Animals
Astrocyte
Brain - cytology
Canavan disease
Canavan Disease - therapy
Cell Differentiation - physiology
Cell Survival
Cell therapy
Cell Transplantation
Disease Models, Animal
Humans
Knockout mouse
Mice
Mice, Knockout
Neural progenitor cell
Oligodendrocyte
Oligodendroglia - cytology
Retrovirus
Stem Cell Transplantation
Stem Cells - cytology
Transfection
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Canavan disease (CD) is an autosomal recessive disorder that leads to spongy degeneration in the white matter of the brain. Aspartoacylase (ASPA) synthesizing cells, oligodendrocytes, are lost in CD. Transplantation of neural progenitor cells (NPCs) offers an interesting therapeutic approach for treating neurodegenerative diseases by replacing the lost cells. Therefore, the NPCs transplantation to the brain of the CD mouse was studied. Injection of mouse NPCs to the striatum and cerebellum of juvenile CD mouse showed numerous BrdU positive cells at 1 month after injection. The same result was also observed in the adult CD mouse brain after 5 weeks of post-transplantation period. The implanted cells differentiated into oligodendrocytes and fibrous astrocytes, as observed using glial cell marker. This is the first report to describe the survival, distribution and differentiation of NPCs within the brain of CD mouse and a first step toward the potential clinical use of cell therapy to treat CD.
ISSN:0165-3806
DOI:10.1016/j.devbrainres.2004.07.003