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Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage.

Abstract：

:Proteolipid protein (PLP) is an integral membrane protein of CNS myelin. Mutations of the X chromosome-linked PLP gene cause glial cell death and myelin deficiency in jimpy mice and other neurological mutants. As part of an attempt to rescue these mutants by transgenic complementation, we generated normal mouse lines expressing autosomal copies of the entire wild-type PLP gene. Surprisingly, increase of the PLP gene dosage in nonmutant mice with only 2-fold transcriptional overexpression results in a novel phenotype characterized by severe hypomyelination and astrocytosis, seizures, and premature death. This demonstrates that precise control of the PLP gene is a critical determinant of terminal oligodendrocyte differentiation. Dysmyelination of PLP transgenic mice provides experimental evidence that Pelizaeus-Merzbacher disease, previously associated with a partial duplication of the human X chromosome, can be caused by doubling of the PLP gene dosage.

journal_name

Neuron

journal_title

Neuron

authors

Readhead C,Schneider A,Griffiths I,Nave KA

doi

10.1016/0896-6273(94)90214-3

subject

Has Abstract

pub_date

1994-03-01 00:00:00

pages

583-95

issue

3

eissn

0896-6273

issn

1097-4199

pii

0896-6273(94)90214-3

journal_volume

12

pub_type

杂志文章

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