Literature DB >> 7388618

Hypomyelinated mutant mice: description of jpmsd and comparison with jp and qk on their present genetic backgrounds.

S Billings-Gagliardi, L H Adcock, M K Wolf.   

Abstract

Hypomyelinated mutant mice are valuable natural animal systems for analysis of CNS myelin development, chemistry and diseases. One mutant, jpmsd, has never received thorough morphological description. We here describe the detailed morphology of jpmsd, compare it with well-studied jp and qk on their present genetic backgrounds, and discuss the genetic histories of all 3 mutants. Region for region, jpmsd has twice as much myelin as jp, but 1/2--1/5 as much as qk. Both jp and jpmsd have scarce oligodendrocytes, rare nodes of Ranvier, clustering of myelin segments, abnormal lipid-filled cells, frequent degenerating cells, and rare distorted myelin profiles. In contrast, qk has abnormally numerous oligodendrocytes, frequent nodes of Ranvier, no obvious myelin clustering, no lipid-filled cells, rare degenerating cells, and frequent abnormal or distorted myelin profiles. jp and jpmsd are quantitatively different, but qualitatively similar. Since its origin, the jpmsd disease has inadvertently been ameliorated by transferring the mutation to a different background. Persistent differences in the remainder of the genome might account for all remaining apparent differences between jp and jpmsd. In contrast, qk is totally dissimilar in morphology and presumably in pathophysiology.

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Year:  1980        PMID: 7388618     DOI: 10.1016/0006-8993(80)91215-9

Source DB:  PubMed          Journal:  Brain Res        ISSN: 0006-8993            Impact factor:   3.252


  8 in total

1.  Brain DNA metabolism in myelin deficient mutant jp, jpmsd and qk mice.

Authors:  S N Shah; R C Johnson
Journal:  Neurochem Res       Date:  1983-12       Impact factor: 3.996

2.  The quakingviable mutation affects qkI mRNA expression specifically in myelin-producing cells of the nervous system.

Authors:  Zifan Lu; Youyi Zhang; Li Ku; Houping Wang; Amir Ahmadian; Yue Feng
Journal:  Nucleic Acids Res       Date:  2003-08-01       Impact factor: 16.971

3.  Noradrenergic neurotransmission in the brain of a convulsive mutant mouse, differences between the cerebral cortex and the brain stem.

Authors:  Y Maurin; S Arbilla; J Dedek; C R Lee; N Baumann; S Z Langer
Journal:  Naunyn Schmiedebergs Arch Pharmacol       Date:  1982-07       Impact factor: 3.000

4.  Minimal role for caspase 12 in the unfolded protein response in oligodendrocytes in vivo.

Authors:  Ramaswamy Sharma; Alexander Gow
Journal:  J Neurochem       Date:  2007-03-30       Impact factor: 5.372

5.  Aberrant splicing of proteolipid protein mRNA in the dysmyelinating jimpy mutant mouse.

Authors:  L D Hudson; J A Berndt; C Puckett; C A Kozak; R A Lazzarini
Journal:  Proc Natl Acad Sci U S A       Date:  1987-03       Impact factor: 11.205

6.  Pelizaeus-Merzbacher disease: an X-linked neurologic disorder of myelin metabolism with a novel mutation in the gene encoding proteolipid protein.

Authors:  S Gencic; D Abuelo; M Ambler; L D Hudson
Journal:  Am J Hum Genet       Date:  1989-09       Impact factor: 11.025

7.  Analysis of myelin proteolipid protein and F0 ATPase subunit 9 in normal and jimpy CNS.

Authors:  J A Benjamins; D M Studzinski; R P Skoff
Journal:  Neurochem Res       Date:  1994-08       Impact factor: 3.996

8.  Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease.

Authors:  A Gow; C M Southwood; R A Lazzarini
Journal:  J Cell Biol       Date:  1998-02-23       Impact factor: 10.539

  8 in total

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