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

Sankar Surendran, Lamya S. Shihabuddin, Jennifer Clarke, Tatyana V. Taksir, Gregory R. Stewart, Geoffrey Parsons, Wendy Yang, Stephen K. Tyring, Kimberlee Michals-Matalon, Reuben Matalon

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)19-27
Number of pages9
JournalDevelopmental Brain Research
Volume153
Issue number1
DOIs
StatePublished - Oct 15 2004
Externally publishedYes

Keywords

  • Astrocyte
  • Canavan disease
  • Cell therapy
  • Knockout mouse
  • Neural progenitor cell
  • Oligodendrocyte
  • Retrovirus

ASJC Scopus subject areas

  • Developmental Neuroscience
  • Developmental Biology

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  • Cite this

    Surendran, S., Shihabuddin, L. S., Clarke, J., Taksir, T. V., Stewart, G. R., Parsons, G., Yang, W., Tyring, S. K., Michals-Matalon, K., & Matalon, R. (2004). Mouse neural progenitor cells differentiate into oligodendrocytes in the brain of a knockout mouse model of Canavan disease. Developmental Brain Research, 153(1), 19-27. https://doi.org/10.1016/j.devbrainres.2004.07.003