Search Criteria: Research Area is "Mouse/Human Gene Homologs: globoid cell leukodystrophy (Krabbe disease)"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 000845 | B6.CE-Galctwi/J | Repository-Cryopreserved |
| The twitcher mouse is a neurological leukodystrophy mutant first observed in 1976 at The Jackson Laboratory. Initially characterized on a mixed C57BL/6J and CE/J background, clinical symptoms were first observed by day 30 and homozygotes did not survive beyond three months of age. (Duchen LW, et. al., 1980) Subsequent backcrosses to C57BL/6J and the generation of a full congenic (> 10 backcrosses) reduced the onset of symptoms to approximately 21 days with death by 40 days. Head tremors and decreased body weight are initial clinical indicators and mice are generally less active than unaffected littermates. Muscle weakness in the hindlimbs is a promiment feature and clinically, the health of the mutants progressively declines until death. There is a significant lack of myelin in the twitcher CNS, along with astrocytic gliosis. The nerves in the PNS are also demyelinated. The mutant CNS and PNS contain multinucleated, periodic acid-Schiff-positive globoid cells. Electron microscopi
..... For more information please see the full descriiption on the strain data sheet | ||
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