Cell Stem Cell, Volume 2, Issue 6, 553-565 5 June 2008

doi:10.1016/j.stem.2008.03.020

Neonatal Chimerization with Human Glial Progenitor Cells Can Both Remyelinate and Rescue the Otherwise Lethally Hypomyelinated Shiverer Mouse

Supplemental Data for Windrem et al.

Document S1. Two Figures (PDF 337 kb)
Movie S1. Untreated P129 (MOV 848 kb)
This untreated 16-week-old shiverer mouse (shi/shi × rag2−/−) manifests the truncal instability and intention tremor typical of shiverers. The mice also develop progressively worsening seizures, hindlimb weakness, and dyscoordinated forward ambulation, such that by 18–19 weeks of age they are substantially impaired, and typically die by 140 days.
Movie S2. Seizure at Night P141 (MOV 919 kb)
This 20-week-old shiverer mouse (shi/shi × rag2−/−) manifests the typical seizure of shiverer mice, comprising a brief period of tonic akinesia followed by a tonic-clonic phase.
Movie S3. Transplant 1 Year (MOV 886 kb)
This 54-week-old shiverer mouse (shi/shi × rag2−/−) was transplanted at birth with 300,000 human glial progenitor cells, as described. It still manifests a truncal wobble characterized by wide-based hind limbs and some truncal instability. Yet its seizures completely abated by 36 weeks, its exploratory activities have recovered to the point of being indistinguishable from wild-type controls, and its tremor has abated to the extent that it no longer freezes on forward movement, allowing essentially normal ambulation.

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