Abnormality in cultured oligodendrocytes and Schwann cells isolated from the twitcher mouse.
Oligodendrocytes and Schwann cells were isolated from the brain and dorsal root ganglia of the twitcher mouse, a murine model of Krabbe's disease (globoid cell leukodystrophy), and grown in tissue culture. Oligodendrocytes were cultured for up to 22 d in vitro and were immunostained with a galactocerebroside antibody, a specific marker for oligodendrocytes. The control oligodendrocytes developed well-branched processes and membrane sheets, whereas the twitcher oligodendrocyte had wirelike processes with no membrane expansion and progressive degeneration. Schwann cells from the twitcher could not extend their processes as long as normal counterparts. The amounts of psychosine in the enriched population of oligodendrocytes and Schwann cells from the twitcher mouse are about 50-fold and 70-fold higher, respectively, than those in the control cells. These data suggest that psychosine may play an important role in the progression of abnormal features of oligodendroglial membrane formation and in the absence of process elongation in Schwann cells in the twitcher mutant.
Ida
H
H
Department of Pediatrics, Tokyo Jikei University School of Medicine, Japan.
Kawame
F
F
Kim
S U
SU
Eto
Y
Y
D000818:Animals; D000911:Antibodies, Monoclonal; D002478:Cells, Cultured; D007150:Immunohistochemistry; D051379:Mice; D008818:Mice, Neurologic Mutants; D009410:Nerve Degeneration; D009422:Nervous System Diseases / Q000503:physiopathology*; D009836:Oligodendroglia / Q000473:pathology / Q000502:physiology*; D011609:Psychosine / Q000378:metabolism; D012583:Schwann Cells / Q000473:pathology / Q000502:physiology*
D000911:Antibodies, Monoclonal; D011609:Psychosine