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 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.
Surendran
Sankar
S
Pediatrics Rm # 3.350, Department of Pediatrics, Childrens Hospital, The University of Texas Medical Branch, Galveston, TX 77555-0359, USA.
Shihabuddin
Lamya S
LS
Clarke
Jennifer
J
Taksir
Tatyana V
TV
Stewart
Gregory R
GR
Parsons
Geoffrey
G
Yang
Wendy
W
Tyring
Stephen K
SK
Michals-Matalon
Kimberlee
K
Matalon
Reuben
R
D000581:Amidohydrolases / Q000235:genetics; D000818:Animals; D001921:Brain / Q000166:cytology*; D017825:Canavan Disease / Q000628:therapy*; D002454:Cell Differentiation / Q000502:physiology*; D002470:Cell Survival; D017690:Cell Transplantation; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D009836:Oligodendroglia / Q000166:cytology*; D033581:Stem Cell Transplantation; D013234:Stem Cells / Q000166:cytology; D014162:Transfection
D000581:Amidohydrolases; C019751:aspartoacylase