Abstract
We have previously established spontaneously immortalized cell lines from long-term cultures of normal adult mouse Schwann cells. Establishment of such Schwann cell lines derived from murine disease models may greatly facilitate studies of the cellular mechanisms of their peripheral nervous system lesions in the relevant diseases. We maintained long-term cultures of Schwann cells derived from dorsal root ganglia and consecutive peripheral nerves of Niemann-Pick disease type C mice (spm/spm, npcnih/npcnih), globoid cell leukodystrophy mice (twitcher), myelin P0 protein-deficient mice (P0-/-), neurofibromatosis type 1 gene (NF1)-deficient mice (Nf1Fcr/+), Sandhoff disease mice (HexB-/-), and Fabry disease mice (Gla-) for 8-10 months, and established spontaneously immortalized cell lines from all these animals. These cell lines showed spindle-shaped Schwann cell morphology and distinct Schwann cell phenotypes and retained genomic and biochemical abnormalities, sufficiently representing the in vivo pathological features of the mutant mice. We have been able to obtain these immortalized Schwann cell lines from mice of ICR, BALB/c, and C57BL strains, and it is likely that the spontaneous immortalization of long-term cultured Schwann cells is a general phenomenon in both normal (wild-type) and mutant mice. These immortalized Schwann cell lines can be useful in studies of nervous system lesions in these mutant mice and relevant human disorders. [J Physiol Sci. 2008;58 Suppl:S37]
