Isolation and morphologic characterization in vitro of Schwann cells of the twitcher mouse.
Schwann cells were isolated and cultured from the twitcher mouse, an authentic model of human globoid cell leukodystrophy. We observed some difference in morphology and initial cell growth between the cells from suckling and adult mice. Initial growth was slower in homozygous and heterozygous twitcher (twi/twi; twi/+) cells than in normal cells (+/+) when cells were cultured from suckling mice. On the other hand, cell growth was initially accelerated in cells from adult twi/twi mice compared with adult twi/+ or +/+ animals. The yield of Schwann cells was much less from adults. In the cells from adult twi/twi, the perikaryon was swollen, and typical inclusions were easily recognized ultrastructurally, in particular after 14 days in vitro. However, only a few globoid cell leukodystrophy inclusions were detected in the cells from suckling twi/twi mice even after 28 days in vitro. The results indicate that Schwann cell pathology can be studied in twi/twi using an in vitro system and that twi/twi Schwann cells from suckling and adult mice have some differences in morphology as well as in the degree of initial growth.
Irino
M
M
Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill.
Suzuki
K
K
D000818:Animals; D002478:Cells, Cultured; D004195:Disease Models, Animal; D005455:Fluorescent Antibody Technique; D006579:Heterozygote; D006720:Homozygote; D066298:In Vitro Techniques; D007965:Leukodystrophy, Globoid Cell / Q000235:genetics / Q000473:pathology*; D051379:Mice; D008818:Mice, Neurologic Mutants; D008855:Microscopy, Electron, Scanning; D012583:Schwann Cells / Q000502:physiology / Q000648:ultrastructure*