Jm. Orian et al., DELAYED AND INCOMPLETE MYELINATION IN A TRANSGENIC MOUSE MUTANT WITH ABNORMAL OLIGODENDROCYTES, Journal of neuroscience research, 50(5), 1997, pp. 809-820
In search of animal models suitable for investigating myelin repair, w
e have analysed myelinogenesis in a transgenic mouse mutant with delay
ed myelination, but with a normal life-span, The 2-50 mutant which car
ries a c-myc gene under the regulation of the myelin basic protein pro
moter has been described previously (Orian et al.: J Neurosci Res 39:6
04-612, 1994), Here we show that appropriate mRNA transcripts and thei
r corresponding protein products are generated, but that the accumulat
ion of these products is delayed in transgenic mice with respect to no
ntransgenic littermates. This phenomenon is associated with aberrant m
yelin and paucity of normal oligodendrocytes, Myelination appears to b
e carried out by abnormal, oligodendrocyte-like cells. We propose that
the primary defect in the 2-50 mutant is an inability to generate the
normal number of mature oligodendrocytes, This mutant represents a no
vel class of mutant in which oligodendrocyte development and myelinati
on can be studied in the absence of interference with a gene for a str
uctural protein of myelin, in an animal with normal survival, It may a
lso represent a new tool to investigate in vivo gliogenesis and regula
tory events bringing about the coordinated regulation of myelin protei
n synthesis. (C) 1997 Wiley-Liss, Inc.