Fate of jimpy-type oligodendrocytes in jimpy heterozygote.

Abstract:

:In the jimpy mutant mouse, as well as in many other animals with mutations in the myelin proteolipid protein (PLP) gene, oligodendrocytes degenerate before their maturation. To analyze whether this degeneration is caused by the loss of function of PLP gene products related to oligodendrocyte maturation/survival acting extrinsically, expression of the PLP gene was investigated in the jimpy heterozygote, in which one-half of the cells are jimpy type and the other half are wild type due to random X-chromosome inactivation. We first showed that jimpy PLP gene expression is normally regulated at the early stages of development in brains of jimpy hemizygotes and heterozygotes, at least to day 2 after birth. However, the great increase in the level of PLP gene transcripts observed in wild-type mouse brain is suppressed in jimpy mouse brain. This increase was also suppressed in the jimpy heterozygote, and by 2 months after birth, very few jimpy-type PLP gene transcripts were detected in heterozygotes. These results indicate that jimpy-type oligodendrocytes cannot survive or are still in the immature stage in the brain of jimpy heterozygotes. Thus, degeneration of jimpy oligodendrocytes is not caused merely by the lack of trophic factors.

journal_name

J Neurochem

authors

Kagawa T,Nakao J,Yamada M,Shimizu K,Hayakawa T,Mikoshiba K,Ikenaka K

doi

10.1046/j.1471-4159.1994.62051887.x

subject

Has Abstract

pub_date

1994-05-01 00:00:00

pages

1887-93

issue

5

eissn

0022-3042

issn

1471-4159

journal_volume

62

pub_type

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