Embryonic stem cell rescue of tremor and ataxia in myelin-deficient shiverer mice
Department of Neurology; Department of Cell Biology
Stem cells; Myelin basic protein; *X-gal histochemistry; Blastocyst injection; Chimera; Oligodendrocytes; Shiverer mice; White matter
Cell Biology | Neurology | Neuroscience and Neurobiology
Transplantation of neural precursor cells has been proposed as a possible approach for replacing missing or damaged central nervous system myelin. Neonatal and adult myelin-deficient shiverer (shi) mice, bearing a mutation of the myelin basic protein (MBP) gene, have been used extensively as hosts for testing cell engraftment, migration, and myelination, but relatively little progress has been made in reversing shi motor deficits. Here we describe a prenatal cell replacement strategy, showing that embryonic stem cells injected into shi blastocyst embryos can generate chimeric mice with strong and widespread immunoreactive MBP expression throughout the brain and a behavioral (motor) phenotype that appears essentially rescued.
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Citation: J Neurol Sci. 2009 Jan 15;276(1-2):133-7. Epub 2008 Nov 8. Link to article on publisher's site