## Abstract The myelinβdeficient (MD) rat has a point mutation in its proteolipid protein (PLP) gene that causes severe dysmyelination and oligodendrocyte cell death. Using an in vitro model, we have shown that MD oligodendrocytes initially differentiate similarly to wildβtype cells, expressing gal
Gene expression and oligodendrocyte development in the myelin deficient rat
β Scribed by Dr. N. L. Nadon; I. D. Duncan
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 898 KB
- Volume
- 41
- Category
- Article
- ISSN
- 0360-4012
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β¦ Synopsis
The proteolipid proteins play a major role in the structure of the CNS myelin sheath, but they have also been implicated in the oligodendrocyte development leading to myelination. Mutations in the PLP gene result in severe dysmyelination and a paucity of mature oligodendrocytes. The myelin deficient (md) rat, carrying a Thr75+Pro substitution present in both isoforms of proteolipid protein (PLP and DM20), is the most severely affected of the PLP mutants described to date. The expression of myelin associated genes was quantitated to determine the effect of the mutation on oligodendrocyte development in vivo. At 5 days postnatal, gene expression in the md rat approximated that in age-matched control rats, but as they matured, there was a progressive inhibition of gene expression in the md rats. The genes expressed late in the myelination program (PLP and MBP) were affected more dramatically than those expressed earlier in oligodendrocyte development (CNP and GPDH). The results indicate that the later stages of oligodendrocyte maturation and myelin elaboration are inhibited.
π SIMILAR VOLUMES
A point mutation in exon 3 of the proteolipid protein (PLP) gene of the myelin-deficient (md) rat leads to a failure of oligodendrocyte maturation and early death of oligodendrocytes, resulting in dysmyelination. It has been suggested that an alternative-splice isoform of PLP, known as DM-20, might
## Abstract We have shown previously that myelinβforming oligodendrocytes express the protein tyrosine phosphatase SHPβ1 and that myelin formation was decreased in SHPβ1βdeficient motheaten mice compared to that in normal littermates. These studies suggested a potential importance for SHPβ1 in olig
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We have studied gene expression of neuroglial cell markers in the myelin-deficient (md) rat brain during postnatal development. Northern blots and slot blots of poly(A)+ RNA from developing brain were sequentially probed with cDNAs specific for the oligodendrocyte markers glycerol phosphate dehydrog
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