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Fate of Jimpy‐Type Oligodendrocytes in Jimpy Heterozygote
Author(s) -
Kagawa Tetsushi,
Nakao Junji,
Yamada Masanobu,
Shimizu Keiji,
Hayakawa. Toru,
Mikoshiba Katsuhiko,
Ikenaka Kazuhiro
Publication year - 1994
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1046/j.1471-4159.1994.62051887.x
Subject(s) - heterozygote advantage , biology , wild type , oligodendrocyte , myelin , genetics , gene , mutant , allele , endocrinology , central nervous system
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 Xchromosome 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.