jimpy mice


Summary: Myelin-deficient mutants which are from the inbred Tabby-Jimpy strain.

Top Publications

  1. Baracskay K, Duchala C, Miller R, Macklin W, Trapp B. Oligodendrogenesis is differentially regulated in gray and white matter of jimpy mice. J Neurosci Res. 2002;70:645-54 pubmed
    ..These data provide additional evidence that oligodendrogenesis is differentially regulated in white matter and gray matter and implicate PLP/DM20 as a modulator of these differences. ..
  2. Gow A, Friedrich V, Lazzarini R. Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport. J Neurosci Res. 1994;37:574-83 pubmed
  3. Nadon N, Arnheiter H, Hudson L. A combination of PLP and DM20 transgenes promotes partial myelination in the jimpy mouse. J Neurochem. 1994;63:822-33 pubmed
    ..The transgenes were bred into jimpy mice, and the effect of the transgenes on the dysmyelinating phenotype was analyzed...
  4. Gow A, Lazzarini R. A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease. Nat Genet. 1996;13:422-8 pubmed
    ..Moreover, we show that transport-competent mutant DM20s facilitate trafficking of cognate PLPs and hence may influence disease severity...
  5. Kobayashi H, Hoffman E, Marks H. The rumpshaker mutation in spastic paraplegia. Nat Genet. 1994;7:351-2 pubmed
  6. Gardinier M, Macklin W, Diniak A, Deininger P. Characterization of myelin proteolipid mRNAs in normal and jimpy mice. Mol Cell Biol. 1986;6:3755-62 pubmed
    ..This size difference was seen in the two major PLP mRNAs, and it did not result from a loss of polyadenylation of these mRNAs...
  7. Vela J, Dalmau I, Acarin L, Gonzalez B, Castellano B. Microglial cell reaction in the gray and white matter in spinal cords from jimpy mice. An enzyme histochemical study at the light and electron microscope level. Brain Res. 1995;694:287-98 pubmed
    ..Compared to those of normal littermates, the spinal cords of jimpy mice showed an intense microglial cell reaction in white and gray matter, as revealed by quantitative analysis and ..
  8. Vela J, Dalmau I, Gonzalez B, Castellano B. The microglial reaction in spinal cords of jimpy mice is related to apoptotic oligodendrocytes. Brain Res. 1996;712:134-42 pubmed
    ..Compared to those of normal littermates, the spinal cords of jimpy mice showed a significantly higher number of apoptotic cells...
  9. Ghandour M, Skoff R. Expression of galactocerebroside in developing normal and jimpy oligodendrocytes in situ. J Neurocytol. 1988;17:485-98 pubmed
    ..These morphological changes are observed for both normal and jimpy mice and they parallel those described in vitro...

More Information


  1. Rosenfeld J, Friedrich V. Oligodendrocyte production and myelin recovery in heterozygous jimpy mice: an autoradiographic study. Int J Dev Neurosci. 1986;4:179-87 pubmed
    ..Further study of the cellular interactions which trigger oligodendrocyte production and myelin recovery in the jimpy heterozygote may be relevant to remyelination in other disease states, including those affecting humans...
  2. Vela J, Gonzalez B, Castellano B. Understanding glial abnormalities associated with myelin deficiency in the jimpy mutant mouse. Brain Res Brain Res Rev. 1998;26:29-42 pubmed
    ..The accurate knowledge of the significance of PLP and its regulation during development must be of vital importance in order to understand glial abnormalities in jimpy...
  3. Skoff R. Programmed cell death in the dysmyelinating mutants. Brain Pathol. 1995;5:283-8 pubmed
    ..PCD of cells in normal development involving DNA fragmentation has been previously described and our results support that conclusion.(ABSTRACT TRUNCATED AT 250 WORDS)..
  4. Best T, Skoff R, Bartlett W. Astroglial plasticity in hemizygous and heterozygous jimpy mice. Int J Dev Neurosci. 1988;6:39-57 pubmed
    ..These and other observations suggest that the glial hypertrophy in the young mosaic is temporary and that regression and reorganization of glial processes takes place as myelination proceeds...
  5. Bartlett W, Li X, Connor J. Expression of transferrin mRNA in the CNS of normal and jimpy mice. J Neurochem. 1991;57:318-22 pubmed
    ..It is concluded from this study that the majority of the transferrin mRNA in the mouse brain is expressed by and/or requires the presence of a mature oligodendrocytic population...
  6. Fanarraga M, Sommer I, Griffiths I, Montague P, Groome N, Nave K, et al. Oligodendrocyte development and differentiation in the rumpshaker mutation. Glia. 1993;9:146-56 pubmed
    ..The mutation in jprsh at residue 186 suggests that this region, which is common to PLP and DM-20, is not critical for this latter function...
  7. Schneider A, Griffiths I, Readhead C, Nave K. Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein. Proc Natl Acad Sci U S A. 1995;92:4447-51 pubmed
    ..mutation in the gene for myelin proteolipid protein (PLP) underlies oligodendrocyte death and dysmyelination in jimpy mice, an accurate model for Pelizaeus-Merzbacher disease...
  8. Knapp P, Dutta S, Skoff R. Differences in levels of neuroglial cell death in jimpy male mice and carrier females. Dev Neurosci. 1990;12:145-52 pubmed
    ..Even if this occurs, the data still support the idea that the phenotype of genetically jp cells can be favorably altered by the mosaic environment. ..
  9. Bongarzone E, Foster L, Byravan S, Schonmann V, Campagnoni A. Temperature-dependent regulation of PLP/DM20 and CNP gene expression in two conditionally-immortalized jimpy oligodendrocyte cell lines. Neurochem Res. 1997;22:363-72 pubmed
    ..Our results provide two cell models for in vitro investigations into the nature of the jimpy mutation at several cellular and molecular levels. ..
  10. Yamamoto T, Nanba E, Zhang H, Sasaki M, Komaki H, Takeshita K. Jimpy(msd) mouse mutation and connatal Pelizaeus-Merzbacher disease. Am J Med Genet. 1998;75:439-40 pubmed
  11. Fannon A, Moscarello M. Myelin basic protein is affected by reduced synthesis of myelin proteolipid protein in the jimpy mouse. Biochem J. 1990;268:105-10 pubmed
    ..This suggested that MBPs from early-myelinating and jimpy mice did not react to MBP antisera because C8 was the major charge isomer in these animals...
  12. Lachapelle F, Lapie P, Campagnoni A, Gumpel M. Oligodendrocytes of the jimpy phenotype can be partially restored by environmental factors in vivo. J Neurosci Res. 1991;29:235-43 pubmed
    ..Finally, comparison of the ratio of successful outcomes observed following embryonic vs. newborn jp donor tissue, strongly suggests a partial or total normalization of jp embryonic ODCs survival by a more mature shi environment. ..
  13. Nadon N, Draeger K. Genomic DNA analysis from mouse toe lysates. Transgenic Res. 1996;5:209-11 pubmed
    ..This can be done as early as 6 days of age, minimizing trauma to the mice and allowing assays to be completed long before weaning. ..
  14. Mateu L, Luzzati V, Vonasek E, Borgo M, Lachapelle F. Order-disorder phenomena in myelinated nerve sheaths. VI. The effects of quaking, jimpy and shiverer mutations: an X-ray scattering study of mouse sciatic and optic nerves. J Mol Biol. 1996;256:319-29 pubmed
  15. Birling M, Nussbaum F, Nussbaum J. A new oligodendrocyte specific plasma membrane surface protein identified by a monoclonal antibody produced in vitro. J Neurosci Res. 1994;38:538-50 pubmed
    ..A distinct protein doublet of 53-56 kDa was also observed. It is postulated that this antigen may play an essential role in myelin formation and could be a possible target in diseases restricted to CNS myelin. ..
  16. Knapp P, Booth C, Skoff R. The pH of jimpy glia is increased: intracellular measurements using fluorescent laser cytometry. Int J Dev Neurosci. 1993;11:215-26 pubmed
    ..The jimpy mutation lies in the gene which codes for myelin proteolipid protein, and the brains and spinal cords of jimpy mice contain little myelin and no measurable proteolipid protein...
  17. Ono J, Harada K, Takahashi M, Maeda M, Ikenaka K, Sakurai K, et al. Differentiation between dysmyelination and demyelination using magnetic resonance diffusional anisotropy. Brain Res. 1995;671:141-8 pubmed
    ..In jimpy mice, diffusional anisotropy of optic nerve did not show a significant difference compared to age-matched control ..
  18. Karthigasan J, Evans E, Vouyiouklis D, Inouye H, Borenshteyn N, Ramamurthy G, et al. Effects of rumpshaker mutation on CNS myelin composition and structure. J Neurochem. 1996;66:338-45 pubmed
    ..Our findings thus demonstrate that the biochemical abnormalities in the jprsh mutant correlate with a wider periodicity and less stable packing of the myelin. ..
  19. Black J, Fields R, Waxman S. Macromolecular structure of axonal membrane in the optic nerve of the jimpy mouse. J Neuropathol Exp Neurol. 1988;47:588-98 pubmed
    The macromolecular structure of the axon membrane in 26-28-day-old Jimpy mice and control optic nerve were examined with quantitative freeze-fracture electron microscopy...
  20. Mathis C, Denisenko Nehrbass N, Girault J, Borrelli E. Essential role of oligodendrocytes in the formation and maintenance of central nervous system nodal regions. Development. 2001;128:4881-90 pubmed
    ..1 K(+) channels) axonal markers, was studied in the brain of MBP-TK and jimpy mice. In MBP-TK transgenic mice, oligodendrocyte ablation was selectively induced by FIAU treatment before and during ..
  21. Sinclair A, Raz Y, Kirschner D, Villa Komaroff L, Wolf M, Billings Gagliardi S. Shiverer jimpy double mutant mice. V. Correlation of genotype and myelin proteins. Dev Neurosci. 1991;13:138-42 pubmed
    ..These results differ from an earlier report of both MBP and PLP in the brains of presumed shi*jp animals. ..
  22. Denisenko Nehrbass N, Goutebroze L, Galvez T, Bonnon C, Stankoff B, Ezan P, et al. Association of Caspr/paranodin with tumour suppressor schwannomin/merlin and beta1 integrin in the central nervous system. J Neurochem. 2003;84:209-21 pubmed
    ..Since integrin beta1 and schwannomin do not appear to be enriched in paranodes they may be quantitatively minor partners of paranodin in these regions and/or be associated with paranodin at other locations. ..
  23. Campagnoni A, Campagnoni C, Bourre J, Jacque C, Baumann N. Cell-free synthesis of myelin basic proteins in normal and dysmyelinating mutant mice. J Neurochem. 1984;42:733-9 pubmed
    ..They also indicate that in the jimpy mutant the synthesis of the four BPs is affected to differing extents; thus, the mutant cannot be easily characterized as either an "assembly" or "synthesis" defect. ..
  24. Kagawa T, Nakao J, Yamada M, Shimizu K, Hayakawa T, Mikoshiba K, et al. Fate of jimpy-type oligodendrocytes in jimpy heterozygote. J Neurochem. 1994;62:1887-93 pubmed
    ..Thus, degeneration of jimpy oligodendrocytes is not caused merely by the lack of trophic factors. ..
  25. Hudson L, Berndt J, Puckett C, Kozak C, Lazzarini R. Aberrant splicing of proteolipid protein mRNA in the dysmyelinating jimpy mutant mouse. Proc Natl Acad Sci U S A. 1987;84:1454-8 pubmed
    ..We propose that the jimpy mutant suffers a point mutation or the deletion of a few bases in the PLP gene that alters the normal splicing pattern and generates partially deleted PLP transcripts. ..
  26. Corbeil D, Joester A, Fargeas C, Jászai J, Garwood J, Hellwig A, et al. Expression of distinct splice variants of the stem cell marker prominin-1 (CD133) in glial cells. Glia. 2009;57:860-74 pubmed publisher
    ..Given that prominin-1 is thought to act as an organizer of plasma membrane protrusions, they further suggest that a specific prominin-1 splice variant might play a role in morphogenesis and/or maintenance of the myelin sheath. ..
  27. Mikoshiba K, Kohsaka S, Hayakawa T, Takamatsu K, Tsukada Y. Immunohistochemical localization of 2',3'-cyclic nucleotide 3'-phosphodiesterase and myelin basic protein in the central nervous system of the jimpy and the normal mouse. J Neurochem. 1985;44:686-91 pubmed
    ..The immunohistochemical reaction in the jimpy mice showed a similar localization in both CNPase and MBP.
  28. Nadon N, Miller S, Draeger K, Salvaggio M. Myelin proteolipid DM20: evidence for function independent of myelination. Int J Dev Neurosci. 1997;15:285-93 pubmed
    ..We propose a function for DM20 to explain these diverse findings based on the ability of DM20 to form multimeric complexes, and hypothesize that the DM20 complex participates in intracellular molecular transport. ..
  29. Knapp P, Adjan V, Hauser K. Cell-specific loss of kappa-opioid receptors in oligodendrocytes of the dysmyelinating jimpy mouse. Neurosci Lett. 2009;451:114-8 pubmed publisher
    ..This paper describes a severe reduction in expression of kappa-opioid receptors (KOP) in oligodendrocytes of jimpy mice. A cell-specific reduction of >90% is apparent by 5 days of age...
  30. Verity A, Levine M, Campagnoni A. Gene expression in the jimpy mutant: evidence for fewer oligodendrocytes expressing myelin protein genes and impaired translocation of myelin basic protein mRNA. Dev Neurosci. 1990;12:359-72 pubmed
    ..Rather, they suggest that the inability of jimpy mice to incorporate MBP into myelin may be due, in part, to a disruption in the translocation of MBP mRNAs within the ..
  31. Knapp P, Benjamins J, Skoff R. Epigenetic factors up-regulate expression of myelin proteins in the dysmyelinating jimpy mutant mouse. J Neurobiol. 1996;29:138-50 pubmed
    ..These results also highlight the importance of environmental factors in controlling OL phenotype. ..
  32. Feutz A, Bellomi I, Allinquant B, Schladenhaufen Y, Ghandour M. Isolation and characterization of defective jimpy oligodendrocytes in culture. J Neurocytol. 1995;24:865-77 pubmed
    ..In addition, jimpy oligodendrocytes remain unresponsive to basic fibroblast growth factor treatment while a similar treatment stimulates the proliferation of normal oligodendrocytes. ..
  33. Ikenaka K, Furuichi T, Iwasaki Y, Moriguchi A, Okano H, Mikoshiba K. Myelin proteolipid protein gene structure and its regulation of expression in normal and jimpy mutant mice. J Mol Biol. 1988;199:587-96 pubmed
    ..Therefore, it is likely that a mutation, presumably existing within the jimpy PLP gene, caused the skipping of exon 5 and directly affected the mRNA level. ..
  34. Yuan J, McCartney D, Monge M, Espinosa de los Monteros A, Zalc B, de Vellis J, et al. Glycerophosphorylcholine phosphocholine phosphodiesterase activity in cultured oligodendrocytes, astrocytes, and central nervous tissue of dysmyelinating rodent mutants. J Neurosci Res. 1992;31:68-74 pubmed
  35. Knapp P, Skoff R. A defect in the cell cycle of neuroglia in the myelin deficient jimpy mouse. Brain Res. 1987;432:301-6 pubmed
    ..Therefore, the unusually high L:M ratio indicates that there is an abnormality in some aspect of the jimpy oligodendroglial cell cycle...
  36. Cammer W, Tansey F. Localization of glial cell antigens in the brains of young normal mice and the dysmyelinating mutant mice, jimpy and shiverer. J Neurosci Res. 1988;20:23-31 pubmed
    ..However, the data concerning the jimpy mouse suggested that the few CA-positive cells in the corpus callosum of that mutant could be astrocytes...
  37. Gotow T, Leterrier J, Ohsawa Y, Watanabe T, Isahara K, Shibata R, et al. Abnormal expression of neurofilament proteins in dysmyelinating axons located in the central nervous system of jimpy mutant mice. Eur J Neurosci. 1999;11:3893-903 pubmed
    ..The jimpy mice exhibited a characteristic similarity in neurofilament nature to the myelin-deficient mice in the peripheral ..
  38. Komaki H, Sasaki M, Yamamoto T, Iai M, Takashima S. Connatal Pelizaeus-Merzbacher disease associated with the jimpy(msd) mice mutation. Pediatr Neurol. 1999;20:309-11 pubmed
    ..The mechanism of myelination is partly disturbed by the mutation; therefore jimpy(msd) mice can be used as a suitable model for further studies in connatal Pelizaeus-Merzbacher disease...
  39. Campagnoni C, Garbay B, Micevych P, Pribyl T, Kampf K, Handley V, et al. DM20 mRNA splice product of the myelin proteolipid protein gene is expressed in the murine heart. J Neurosci Res. 1992;33:148-55 pubmed
    ..These results indicate that the PLP gene is expressed in tissues other than brain and support the concept that products of the PLP gene may have some biological role other than as structural components of myelin...
  40. Ghandour M, Feutz A, Jalabi W, Taleb O, Bessert D, Cypher M, et al. Trafficking of PLP/DM20 and cAMP signaling in immortalized jimpy oligodendrocytes. Glia. 2002;40:300-11 pubmed
    ..The abnormal insertion of jimpy PLP/DM20 into the plasma membrane may be the basis for the lack of cell signaling and abnormal resting potential in jimpy oligodendrocytes...
  41. Lachapelle F, Lapie P, Gansmuller A, Villarroya H, Gumpel M. Patchy myelination pattern in the jimpy mouse brain: immunohistochemical study. Glia. 1990;3:375-84 pubmed
  42. Knapp P, Maderspach K, Hauser K. Endogenous opioid system in developing normal and jimpy oligodendrocytes: mu and kappa opioid receptors mediate differential mitogenic and growth responses. Glia. 1998;22:189-201 pubmed
    ..If opiate receptors are expressed by OLs in vivo, their pharmacological manipulation might provide a novel pathway for modulating OL and myelin production both during development and in demyelinated conditions...
  43. Baba H, Akita H, Ishibashi T, Inoue Y, Nakahira K, Ikenaka K. Completion of myelin compaction, but not the attachment of oligodendroglial processes triggers K(+) channel clustering. J Neurosci Res. 1999;58:752-64 pubmed
    ..In addition, postsynaptic density protein-95 (PSD-95) or its highly related protein was found colocalized with K(+) channels, suggesting that it may interact with K(+) channels to form clusters at juxtaparanodal regions...
  44. Kuchler S, Zanetta J, Zaepfel M, Badache A, Sarlieve L, Gumpel M, et al. Endogenous cerebellar soluble lectin and its ligands in central nervous system myelin of quaking and jimpy mutant mice. Dev Neurosci. 1990;12:382-97 pubmed
    ..The nature of the low-Mr glycoproteins absent in quaking and jimpy mice is discussed in relation to previous reports on myelin glycoproteins...
  45. Skoff R, Saluja I, Bessert D, Yang X. Analyses of proteolipid protein mutants show levels of proteolipid protein regulate oligodendrocyte number and cell death in vitro and in vivo. Neurochem Res. 2004;29:2095-103 pubmed
    ..The regulation of apoptosis by PLP gene expression occurs independently of myelination, indicating that the PLP gene has multiple primary functions...
  46. Vela J, Hidalgo J, Gonzalez B, Castellano B. Induction of metallothionein in astrocytes and microglia in the spinal cord from the myelin-deficient jimpy mouse. Brain Res. 1997;767:345-55 pubmed
    ..The occurrence of MT induction in relation to reactive astrocytes and microglia, and its role in neuropathological conditions is discussed...
  47. Readhead C, Hood L. The dysmyelinating mouse mutations shiverer (shi) and myelin deficient (shimld). Behav Genet. 1990;20:213-34 pubmed
    ..The gene encoding MBP has been both duplicated and inverted. Transgenic shimld/shimld mice with the wild-type MBP transgene have normal phenotypes...
  48. Billings Gagliardi S, Kerner A, Kirschner D, Wolf M. Shiverer jimpy double mutant mice. III. Comparison of shimld*jpmsd and shi*jp phenotypes demonstrates dissimilar interactions of allelic mutations. Brain Res. 1987;388:199-214 pubmed
  49. Koeppen A, Barron K, Csiza C, Greenfield E. Comparative immunocytochemistry of Pelizaeus-Merzbacher disease, the jimpy mouse, and the myelin-deficient rat. J Neurol Sci. 1988;84:315-27 pubmed
    ..The lack of oligodendrocytes in jp/Y and md/Y could also be confirmed by immunocytochemistry for transferrin...
  50. Feutz A, Pham Dinh D, Allinquant B, Miehe M, Ghandour M. An immortalized jimpy oligodendrocyte cell line: defects in cell cycle and cAMP pathway. Glia. 2001;34:241-52 pubmed
  51. DasGupta S, Everhart M, Bhat N, Hogan E. Neutral monoglycosylceramides in rat brain: occurrence, molecular expression and developmental variation. Dev Neurosci. 1997;19:152-61 pubmed
    ..A reduction in brain FMC concentrations along with GalCer in murine genetic dysmyelinating disorders (jimpy and quaking) further supports the conclusion that they are myelin constituents...
  52. Vela Hernández J, Dalmau I, Gonzalez B, Castellano B. Abnormal expression of the proliferating cell nuclear antigen (PCNA) in the spinal cord of the hypomyelinated Jimpy mutant mice. Brain Res. 1997;747:130-9 pubmed
    ..The expression of PCNA in relation to the proliferative capability and possible cell cycle abnormalities of the different glial cell types in Jimpy is discussed...
  53. Boulias C, Moscarello M. ADP-ribosylation of myelin basic proteins isolated from normal and mutant mouse brains. Neuroreport. 1990;1:221-4 pubmed
  54. Nishiyama A. Glial progenitor cells in normal and pathological states. Keio J Med. 1998;47:205-8 pubmed
    ..In the spinal cords of jimpy mice at postnatal day 18, there was a three- to six-fold increase in the number of NG2+ glial cells that had ..
  55. Kanashiro M, Naritomi H, Sasaki M, Tsuji S, Miyake Y. Non-invasive 31P NMR study on the development of brain membrane of gerbils and jimpy mice, a myelin-deficient mutant. Physiol Chem Phys Med NMR. 1990;22:141-54 pubmed
    ..Furthermore, the value of T1 of the membrane component was estimated to be 0.9 sec, which was in good agreement with previously reported values for excised brain...
  56. Hatfield J, Skoff R. GFAP immunoreactivity reveals astrogliosis in females heterozygous for jimpy. Brain Res. 1982;250:123-31 pubmed
    ..The results of the present study show that the retardation of myelin formation is accompanied by an astroglial response and that the intensity of the gliosis closely parallels the extent of the hypomyelination...
  57. Bartlett W, Knapp P, Skoff R. Glial conditioned medium enables jimpy oligodendrocytes to express properties of normal oligodendrocytes: production of myelin antigens and membranes. Glia. 1988;1:253-9 pubmed
    ..The dramatic effect of normal glial conditioned medium on jp oligodendrocytes suggests that the steps leading to hypomyelination in jp are exceedingly complex and may involve glial-glial interactions...
  58. Doolittle D, Baumann N, Chernoff G. Allelism of two myelin deficiency mutations in the mouse. J Hered. 1981;72:285 pubmed
  59. Bolivar V, Brown R. Selective retrieval of jimpy mutant pups over normal male littermates by lactating female B6CBACa-Aw-J/A-Ta jp mice. Behav Genet. 1995;25:75-80 pubmed
    ..We are thus left with the paradox that the mother appears to "prefer" the mutant pup to the normal littermate, despite its smaller size, reduced ultrasonic vocalization rate, and compromised fitness...
  60. Knapp P, Skoff R. Jimpy mutation affects astrocytes: lengthening of the cell cycle in vitro. Dev Neurosci. 1993;15:31-6 pubmed
    ..Since there is no evidence that astrocytes make myelin proteolipid protein, the results support previous studies which suggest that this gene may code for other proteins playing an important role in the development of many cell types...
  61. Li X, Bartlett W. Developmental expression of glial fibrillary acidic protein and glutamine synthetase mRNAs in normal and jimpy mice. Brain Res Mol Brain Res. 1991;9:313-7 pubmed
    ..fibrillary acidic protein (GFAP) and glutamine synthetase (GS) mRNAs were examined the cerebra of normal and jimpy mice. The relative abundance of GFAP and GS mRNAs increased rapidly in the CNS of normal mice during the first two ..
  62. Benjamins J, Studzinski D, Skoff R, Nedelkoska L, Carrey E, Dyer C. Recovery of proteolipid protein in mice heterozygous for the jimpy gene. J Neurochem. 1989;53:279-86 pubmed
    ..abstract truncated at 250 words)..
  63. Gardinier M, Macklin W. Myelin proteolipid protein gene expression in jimpy and jimpy(msd) mice. J Neurochem. 1988;51:360-9 pubmed
    ..Thus, jpmsd mice are capable of synthesizing normal PLP and DM20 protein; however, the PLP gene defect has affected the normal developmental pattern of expression for these two proteins...
  64. Hulet S, Menzies S, Connor J. Ferritin binding in the developing mouse brain follows a pattern similar to myelination and is unaffected by the jimpy mutation. Dev Neurosci. 2002;24:208-13 pubmed
    ..may be involved in the induction of ferritin-binding proteins on oligodendrocytes, brains from the myelin mutant jimpy mice and unaffected littermates were examined at postnatal days 16-18...
  65. Montague P, Kirkham D, McCallion A, Davies R, Kennedy P, Klugmann M, et al. Reduced levels of a specific myelin-associated oligodendrocytic basic protein isoform in shiverer myelin. Dev Neurosci. 1999;21:36-42 pubmed
    ..Our data suggest that incorporation of this MOBP isoform into shiverer myelin may be influenced by the presence of MBP or be a consequence of a disrupted MDL...
  66. Shiota C, Ikenaka K, Mikoshiba K. Developmental expression of myelin protein genes in dysmyelinating mutant mice: analysis by nuclear run-off transcription assay, in situ hybridization, and immunohistochemistry. J Neurochem. 1991;56:818-26 pubmed
    ..The number of oligodendrocytes expressing PLP mRNA was drastically reduced in jimpy mice. MBP mRNA in jimpy mice is localized preferentially in oligodendroglial cell bodies, a result suggesting that ..
  67. Gordon M, Kumar S, Espinosa de los Monteros A, de Vellis J. Ontogeny of glycerol phosphate dehydrogenase-positive oligodendrocytes in rat brain. Impaired differentiation of oligodendrocytes in the myelin deficient mutant rat. Int J Dev Neurosci. 1992;10:243-53 pubmed
    ..These results suggest that glial cells, like neurons, may be generated in excessive numbers, and some subsequently die, as a normal concomitant of development...
  68. Peyron F, Timsit S, Thomas J, Kagawa T, Ikenaka K, Zalc B. In situ expression of PLP/DM-20, MBP, and CNP during embryonic and postnatal development of the jimpy mutant and of transgenic mice overexpressing PLP. J Neurosci Res. 1997;50:190-201 pubmed
  69. Fuss B, Baba H, Phan T, Tuohy V, Macklin W. Phosphodiesterase I, a novel adhesion molecule and/or cytokine involved in oligodendrocyte function. J Neurosci. 1997;17:9095-103 pubmed
    ..These data together implicate the importance of p421.HB/PD-Ialpha in oligodendrocyte function, possibly through cell-cell and/or cell-extracellular matrix recognition...
  70. Nave K, Lai C, Bloom F, Milner R. Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing. Proc Natl Acad Sci U S A. 1986;83:9264-8 pubmed
    ..We conclude that a mutation in the gene for PLP that leads to an incorrectly spliced RNA transcript is the primary defect of this genetic disorder...
  71. Fannon A, Mastronardi F, Moscarello M. Isolation and identification of proteolipid proteins in jimpy mouse brain. Neurochem Res. 1994;19:1005-12 pubmed
  72. Gencic S, Hudson L. Conservative amino acid substitution in the myelin proteolipid protein of jimpymsd mice. J Neurosci. 1990;10:117-24 pubmed
    ..The accelerated turnover of oligodendrocytes in both mouse mutants suggests a function for PLP/DM20 in oligodendrocyte differentiation distinct from the role of these proteolipid proteins as structural components of the myelin sheath...
  73. Tvrdik P, Westerberg R, Silve S, Asadi A, Jakobsson A, Cannon B, et al. Role of a new mammalian gene family in the biosynthesis of very long chain fatty acids and sphingolipids. J Cell Biol. 2000;149:707-18 pubmed
    ..Our results strongly implicate this new mammalian gene family in tissue-specific synthesis of very long chain fatty acids and sphingolipids...
  74. Le Goascogne C, Eychenne B, Tonon M, Lachapelle F, Baumann N, Robel P. Neurosteroid progesterone is up-regulated in the brain of jimpy and shiverer mice. Glia. 2000;29:14-24 pubmed
    ..The role of PROG in myelin repair is discussed...
  75. Campagnoni A, Verdi J, Verity A, Amur Umarjee S, Byravan S. Posttranscriptional regulation of myelin protein gene expression. Ann N Y Acad Sci. 1991;633:178-88 pubmed
    ..We have also determined that the protein synthetic step modulated by the steroids is chain initiation, enhancing the rate at which new ribosomal subunits bind to the MBP mRNAs...
  76. Macklin W, Gardinier M, King K, Kampf K. An AG----GG transition at a splice site in the myelin proteolipid protein gene in jimpy mice results in the removal of an exon. FEBS Lett. 1987;223:417-21 pubmed
    The myelin proteolipid protein gene was characterized in jimpy mice to identify the specific mutation that produces dysmyelination, oligodendrocyte cell death, and death of the animal by 30 days of age...
  77. Gow A, Southwood C, Lazzarini R. Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J Cell Biol. 1998;140:925-34 pubmed
    ..Finally, we define a hypothesis that accounts for pathogenesis in most PMD patients and animal models of this disease and, moreover, can be used to develop potential therapeutic strategies for ameliorating the disease phenotype...
  78. Nave K, Lai C, Bloom F, Milner R. Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin. Proc Natl Acad Sci U S A. 1987;84:5665-9 pubmed
    ..DM-20 mRNA is expressed in rat brain with approximately 50% abundance relative to PLP mRNA and appears to be developmentally coregulated...
  79. Bignami A, Perides G. Glial hyaluronate-binding protein in dysmyelinating mice mutants: jimpy, quaking and shiverer. Acta Neuropathol. 1989;79:326-9 pubmed
    ..The findings suggest that GHAP expression by astrocytes is not induced by myelination and that white matter astrocytes constitute a distinct glial population...
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    ..Finally, shi/shi mutants may have a delayed synthesis of MAG. The data are discussed in the light of recent observations concerning the synthesis of myelin proteins and their proposed role in myelin assembly...
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    The corticospinal projection was examined in dysmyelinated, jimpy mice and in unaffected littermates following cortical injections of either wheat germ agglutinin conjugated to horseradish peroxidase or biocytin...