Literature DB >> 10884306

Early onset of axonal degeneration in double (plp-/-mag-/-) and hypomyelinosis in triple (plp-/-mbp-/-mag-/-) mutant mice.

T Uschkureit1, O Sporkel, J Stracke, H Bussow, W Stoffel.   

Abstract

Double (plp-/-mag-/-) and triple (plp-/-mbp-/-mag-/-) null-allelic mouse lines deficient in proteolipid protein (PLP), myelin-associated glycoprotein (MAG), and myelin basic protein (MBP) were generated and characterized genetically, biochemically, and morphologically including their behavioral capacities. The plp-/-mag-/- mutant develops a rapidly progressing axon degeneration in CNS with severe cognitive and motor coordinative deficits but has a normal longevity. CNS axons of the plp-/-mbp-/-mag-/- mouse are hypomyelinated and ensheathed by "pseudomyelin" with disturbed protein and complex lipid composition. The shiverer trait in the plp-/-mbp-/-mag-/- similar to the plp-/-mbp-/- mutant is significantly ameliorated, and its lifespan is considerably prolonged. The longevity of these dysmyelinosis mouse mutants recommends them as suitable models for the long-term evaluation of stem cell therapeutic strategies.

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Year:  2000        PMID： 10884306      PMCID： PMC6772331     

Source DB:  PubMed          Journal:  J Neurosci        ISSN： 0270-6474            Impact factor:   6.167


  53 in total

1.  A rapid method of total lipid extraction and purification.

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Journal:  Can J Biochem Physiol       Date:  1959-08

2.  Differential splicing of MAG transcripts during CNS and PNS development.

Authors:  M B Tropak; P W Johnson; R J Dunn; J C Roder
Journal:  Brain Res       Date:  1988-09       Impact factor: 3.252

3.  Brain lipid composition of the shiverer mouse: (genetic defect in myelin development).

Authors:  T D Bird; D F Farrell; S M Sumi
Journal:  J Neurochem       Date:  1978-07       Impact factor: 5.372

4.  Identification of a new exon in the myelin proteolipid protein gene encoding novel protein isoforms that are restricted to the somata of oligodendrocytes and neurons.

Authors:  E R Bongarzone; C W Campagnoni; K Kampf; E C Jacobs; V W Handley; V Schonmann; A T Campagnoni
Journal:  J Neurosci       Date:  1999-10-01       Impact factor: 6.167

5.  Motor function analysis of myelin mutant mice using a rotarod.

Authors:  P L Kuhn; E Petroulakis; G A Zazanis; R D McKinnon
Journal:  Int J Dev Neurosci       Date:  1995-11       Impact factor: 2.457

6.  Gangliosides in the myelin fraction of developing rats.

Authors:  K Suzuki; S E Poduslo; W T Norton
Journal:  Biochim Biophys Acta       Date:  1967-10-02

7.  Differential expression of MAG isoforms during development.

Authors:  L Pedraza; A B Frey; B L Hempstead; D R Colman; J L Salzer
Journal:  J Neurosci Res       Date:  1991-06       Impact factor: 4.164

8.  Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP.

Authors:  T Weimbs; W Stoffel
Journal:  Biochemistry       Date:  1992-12-15       Impact factor: 3.162

9.  Molecular cloning and primary structure of myelin-associated glycoprotein.

Authors:  M Arquint; J Roder; L S Chia; J Down; D Wilkinson; H Bayley; P Braun; R Dunn
Journal:  Proc Natl Acad Sci U S A       Date:  1987-01       Impact factor: 11.205

10.  Structure and molecular arrangement of proteolipid protein of central nervous system myelin.

Authors:  W Stoffel; H Hillen; H Giersiefen
Journal:  Proc Natl Acad Sci U S A       Date:  1984-08       Impact factor: 11.205
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  19 in total

1.  Morphological changes of myelin sheaths in rats intracranially injected with apotransferrin.

Authors:  Cecilia B Marta; Pablo Paez; Margarita Lopez; Amanda Pellegrino de Iraldi; Eduardo F Soto; Juana M Pasquini
Journal:  Neurochem Res       Date:  2003-01       Impact factor: 3.996

2.  A novel myelin protein zero transgenic zebrafish designed for rapid readout of in vivo myelination.

Authors:  Marnie A Preston; Lisbet T Finseth; Jennifer N Bourne; Wendy B Macklin
Journal:  Glia       Date:  2019-01-09       Impact factor: 7.452

Review 3.  Inflammatory demyelination is not central to the pathogenesis of multiple sclerosis.

Authors:  Wolfgang Brück
Journal:  J Neurol       Date:  2005-11       Impact factor: 4.849

Review 4.  The Neural Stem Cell Microenvironment: Focusing on Axon Guidance Molecules and Myelin-Associated Factors.

Authors:  Chao-Jin Xu; Jun-Ling Wang; Wei-Lin Jin
Journal:  J Mol Neurosci       Date:  2015-03-11       Impact factor: 3.444

5.  Statistical diffusion tensor histology reveals regional dysmyelination effects in the shiverer mouse mutant.

Authors:  J Michael Tyszka; Carol Readhead; Elaine L Bearer; Robia G Pautler; Russell E Jacobs
Journal:  Neuroimage       Date:  2005-10-05       Impact factor: 6.556

6.  Recovery of myelin after induction of oligodendrocyte cell death in postnatal brain.

Authors:  Walid Jalabi; Nelly Boehm; Daniel Grucker; M Said Ghandour
Journal:  J Neurosci       Date:  2005-03-16       Impact factor: 6.167

7.  Myelin-associated glycoprotein and complementary axonal ligands, gangliosides, mediate axon stability in the CNS and PNS: neuropathology and behavioral deficits in single- and double-null mice.

Authors:  Baohan Pan; Susan E Fromholt; Ellen J Hess; Thomas O Crawford; John W Griffin; Kazim A Sheikh; Ronald L Schnaar
Journal:  Exp Neurol       Date:  2005-09       Impact factor: 5.330

8.  The Integrated UPR and ERAD in Oligodendrocytes Maintain Myelin Thickness in Adults by Regulating Myelin Protein Translation.

Authors:  Shuangchan Wu; Sarrabeth Stone; Klaus-Armin Nave; Wensheng Lin
Journal:  J Neurosci       Date:  2020-09-21       Impact factor: 6.167

Review 9.  Oligodendrocytes: biology and pathology.

Authors:  Monika Bradl; Hans Lassmann
Journal:  Acta Neuropathol       Date:  2009-10-22       Impact factor: 17.088

Review 10.  Myelin in cartilaginous fish.

Authors:  Maria Elena de Bellard
Journal:  Brain Res       Date:  2016-01-14       Impact factor: 3.252
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