neurologic mutants mice


Summary: Mice which carry mutant genes for neurologic defects or abnormalities.

Top Publications

  1. Jiao Y, Yan J, Zhao Y, Donahue L, Beamer W, Li X, et al. Carbonic anhydrase-related protein VIII deficiency is associated with a distinctive lifelong gait disorder in waddles mice. Genetics. 2005;171:1239-46 pubmed
    ..These data indicate that the wdl mouse is a Car8 null mutant and that CAR8 plays a central role in motor control...
  2. Kuo G, Arnaud L, Kronstad O Brien P, Cooper J. Absence of Fyn and Src causes a reeler-like phenotype. J Neurosci. 2005;25:8578-86 pubmed
    ..This implies that Src and Fyn are needed for Reelin-dependent events during brain development...
  3. Ando M, Sawada K, Sakata Haga H, Jeong Y, Takeda N, Fukui Y. Regional difference in corticotropin-releasing factor immunoreactivity in mossy fiber terminals innervating calretinin-immunoreactive unipolar brush cells in vestibulocerebellum of rolling mouse Nagoya. Brain Res. 2005;1063:96-101 pubmed
    ..CRF may alter CR-positive UBC-mediated excitatory pathways in the lobule X of RMN and may disturb functions of the lobule X such as cerebellar adaptation for linear motion of the head...
  4. Sawada K, Hosoi E, Bando M, Sakata Haga H, Lee N, Jeong Y, et al. Differential alterations in expressions of ryanodine receptor subtypes in cerebellar cortical neurons of an ataxic mutant, rolling mouse Nagoya. Neuroscience. 2008;152:609-17 pubmed publisher
    ..Such expressional changes in ryanodine receptor subtypes may be involved in Ca(2+) channel alpha(1A) subunit gene mutation, and may alter regulation of intracellular Ca(2+) concentrations in cerebellar cortical neurons...
  5. Nakamura T, Honda M, Kimura S, Tanabe M, Oda S, Ono H. Taltirelin improves motor ataxia independently of monoamine levels in rolling mouse nagoya, a model of spinocerebellar atrophy. Biol Pharm Bull. 2005;28:2244-7 pubmed
  6. Walter J, Alviña K, Womack M, Chevez C, Khodakhah K. Decreases in the precision of Purkinje cell pacemaking cause cerebellar dysfunction and ataxia. Nat Neurosci. 2006;9:389-97 pubmed
    ..Our data support the hypothesis that the precision of intrinsic pacemaking in Purkinje cells is essential for motor coordination and suggest that K(Ca) channels may constitute a potential therapeutic target in EA2...
  7. Dusart I, Guenet J, Sotelo C. Purkinje cell death: differences between developmental cell death and neurodegenerative death in mutant mice. Cerebellum. 2006;5:163-73 pubmed
  8. Torban E, Wang H, Patenaude A, Riccomagno M, Daniels E, Epstein D, et al. Tissue, cellular and sub-cellular localization of the Vangl2 protein during embryonic development: effect of the Lp mutation. Gene Expr Patterns. 2007;7:346-54 pubmed
  9. Nystuen A, Schwendinger J, Sachs A, Yang A, Haider N. A null mutation in VAMP1/synaptobrevin is associated with neurological defects and prewean mortality in the lethal-wasting mouse mutant. Neurogenetics. 2007;8:1-10 pubmed
    ..Taken together, these data indicate that VAMP1 has a vital role in a subset of central nervous system tissues...

More Information

Publications108 found, 100 shown here

  1. Kodama T, Itsukaichi Nishida Y, Fukazawa Y, Wakamori M, Miyata M, Molnar E, et al. A CaV2.1 calcium channel mutation rocker reduces the number of postsynaptic AMPA receptors in parallel fiber-Purkinje cell synapses. Eur J Neurosci. 2006;24:2993-3007 pubmed
    ..These lines of evidence suggest that even a moderate dysfunction of CaV2.1 Ca2+ channel can cause substantial changes in postsynaptic molecular composition of the PF-PC synapses and dendritic structure of PCs...
  2. Kaja S, van de Ven R, Broos L, Frants R, Ferrari M, van den Maagdenberg A, et al. Characterization of acetylcholine release and the compensatory contribution of non-Ca(v)2.1 channels at motor nerve terminals of leaner Ca(v)2.1-mutant mice. Neuroscience. 2007;144:1278-87 pubmed
    ..2 channels, and hampers that of Ca(v)2.3 channels. This is the first report directly showing at single synapses the deficits and plasticity in transmitter release resulting from the Ln mutation of Cacna1a...
  3. Mi W, Beirowski B, Gillingwater T, Adalbert R, Wagner D, Grumme D, et al. The slow Wallerian degeneration gene, WldS, inhibits axonal spheroid pathology in gracile axonal dystrophy mice. Brain. 2005;128:405-16 pubmed
    ..We conclude that axon degeneration mechanisms are more closely related than previously thought and that a link exists in gad between spheroid pathology and Wallerian degeneration that could hold for other disorders...
  4. Hossain W, Antic S, Yang Y, Rasband M, Morest D. Where is the spike generator of the cochlear nerve? Voltage-gated sodium channels in the mouse cochlea. J Neurosci. 2005;25:6857-68 pubmed
  5. Sonnier L, Le Pen G, Hartmann A, Bizot J, Trovero F, Krebs M, et al. Progressive loss of dopaminergic neurons in the ventral midbrain of adult mice heterozygote for Engrailed1. J Neurosci. 2007;27:1063-71 pubmed
    ..They also suggest that mice lacking only one En1 allele could provide a novel model for the study of diseases associated with progressive DA cell death. ..
  6. Bearer E, Zhang X, Jacobs R. Live imaging of neuronal connections by magnetic resonance: Robust transport in the hippocampal-septal memory circuit in a mouse model of Down syndrome. Neuroimage. 2007;37:230-42 pubmed
    ..This is the first examination of in vivo transport dynamics in this pathway and the first report of elevated transport in DS. ..
  7. Sun X, Takagishi Y, Okabe E, Chishima Y, Kanou Y, Murase S, et al. A novel Caspr mutation causes the shambling mouse phenotype by disrupting axoglial interactions of myelinated nerves. J Neuropathol Exp Neurol. 2009;68:1207-18 pubmed publisher
    ..These abnormalities likely contribute to the neurological phenotype of the mutant mice. ..
  8. Takahashi T, Kobayashi T, Ozaki M, Takamatsu Y, Ogai Y, Ohta M, et al. G protein-activated inwardly rectifying K+ channel inhibition and rescue of weaver mouse motor functions by antidepressants. Neurosci Res. 2006;54:104-11 pubmed
    ..These results provide evidence for in vivo GIRK channel inhibition by a group of antidepressants. ..
  9. Duez H, Duhem C, Laitinen S, Patole P, Abdelkarim M, Bois Joyeux B, et al. Inhibition of adipocyte differentiation by RORalpha. FEBS Lett. 2009;583:2031-6 pubmed publisher
  10. Hunt D, King B, Kahn D, Yamoah E, Shull G, Krubitzer L. Aberrant retinal projections in congenitally deaf mice: how are phenotypic characteristics specified in development and evolution?. Anat Rec A Discov Mol Cell Evol Biol. 2005;287:1051-66 pubmed
  11. Blednov Y, Walker D, Iyer S, Homanics G, Harris A. Mice lacking Gad2 show altered behavioral effects of ethanol, flurazepam and gabaxadol. Addict Biol. 2010;15:45-61 pubmed publisher
    ..These results are consistent with earlier proposals that deletion of Gad2 (on 129N2 background) reduces synaptic GABA but also suggest changes in extrasynaptic receptor function. ..
  12. Ilijic E, Guidotti A, Mugnaini E. Moving up or moving down? Malpositioned cerebellar unipolar brush cells in reeler mouse. Neuroscience. 2005;136:633-47 pubmed
    ..The observed displacement of UBCs in the reeler suggests that they originate from the ventricular zone. ..
  13. Chabot N, Charbonneau V, Laramée M, Tremblay R, Boire D, Bronchti G. Subcortical auditory input to the primary visual cortex in anophthalmic mice. Neurosci Lett. 2008;433:129-34 pubmed publisher
    ..In particular, we find no evidence of a direct projection from the auditory mesencephalon to the cortex in this animal model of blindness. ..
  14. Best T, Siarey R, Galdzicki Z. Ts65Dn, a mouse model of Down syndrome, exhibits increased GABAB-induced potassium current. J Neurophysiol. 2007;97:892-900 pubmed
    ..These results indicate that increased expression of GIRK2 containing channels have functional consequences that likely affect the balance between excitatory and inhibitory neuronal transmission. ..
  15. Wiencken Barger A, Djukic B, Casper K, McCarthy K. A role for Connexin43 during neurodevelopment. Glia. 2007;55:675-86 pubmed
    ..In conclusion, neurodevelopment of Shuffler/Cx43 cKO mice is abnormal, and the observed cellular phenotype may explain behavioral disturbances seen in these animals as well as in humans carrying Cx43 mutations. ..
  16. Vanmierlo T, Bloks V, van Vark van der Zee L, Rutten K, Kerksiek A, Friedrichs S, et al. Alterations in brain cholesterol metabolism in the APPSLxPS1mut mouse, a model for Alzheimer's disease. J Alzheimers Dis. 2010;19:117-27 pubmed publisher
  17. Amura C, Marek L, Winn R, Heasley L. Inhibited neurogenesis in JNK1-deficient embryonic stem cells. Mol Cell Biol. 2005;25:10791-802 pubmed
    ..Thus, a genetic approach using JNK-deficient ES cells reveals a novel role for JNK1 involving repression of Wnt expression in neural differentiation modeled in murine ES cells. ..
  18. Niciu M, Ma X, El Meskini R, Pachter J, Mains R, Eipper B. Altered ATP7A expression and other compensatory responses in a murine model of Menkes disease. Neurobiol Dis. 2007;27:278-91 pubmed
    ..The compensatory changes observed in the neurovascular unit provide insight into the success of copper injections within a defined neurodevelopmental period. ..
  19. Sawada K, Kalam Azad A, Sakata Haga H, Lee N, Jeong Y, Fukui Y. Striking pattern of Purkinje cell loss in cerebellum of an ataxic mutant mouse, tottering. Acta Neurobiol Exp (Wars). 2009;69:138-45 pubmed
    ..1 gene defect is different among Purkinje cell phenotypes of the tottering cerebellum rather than the expression pattern of mutated Cav2.1 channels. This may result in the reproducible parasagittal pattern of Purkinje cell loss. ..
  20. Boufares S, Guastavino J, Larsson K. Restoration of staggerer mouse maternal behavior following long-term breeding selection. Physiol Behav. 1993;53:1151-5 pubmed
  21. Tseng K, Lu K, Chien C. A possible cellular mechanism of neuronal loss in the dorsal root ganglia of Dystonia musculorum (dt) mice. J Neuropathol Exp Neurol. 2006;65:336-47 pubmed
  22. Zhang P, Izrael M, Ainbinder E, Ben Simchon L, Chebath J, Revel M. Increased myelinating capacity of embryonic stem cell derived oligodendrocyte precursors after treatment by interleukin-6/soluble interleukin-6 receptor fusion protein. Mol Cell Neurosci. 2006;31:387-98 pubmed
    ..IL6RIL6 acts as an effective stimulator of the myelinating function of ES cell-derived oligodendrocyte precursors, as observed upon transplantation of the IL6RIL6- pretreated cells into brain slices of MBP-deficient shiverer mice. ..
  23. Kaneko S, Wang J, Kaneko M, Yiu G, Hurrell J, Chitnis T, et al. Protecting axonal degeneration by increasing nicotinamide adenine dinucleotide levels in experimental autoimmune encephalomyelitis models. J Neurosci. 2006;26:9794-804 pubmed
    ..Finally, we demonstrate that delayed NAm treatment is also beneficial to EAE models, pointing to the therapeutic potential of NAm as a protective agent for EAE and perhaps MS patients. ..
  24. Yip Y, Mehta N, Magdaleno S, Curran T, Yip J. Ectopic expression of reelin alters migration of sympathetic preganglionic neurons in the spinal cord. J Comp Neurol. 2009;515:260-8 pubmed publisher
    ..These results show that SPN position can change with location and level of reelin expression. Possible functions of Reelin on SPN migration are discussed. ..
  25. Matsuzaki H, Minabe Y, Nakamura K, Suzuki K, Iwata Y, Sekine Y, et al. Disruption of reelin signaling attenuates methamphetamine-induced hyperlocomotion. Eur J Neurosci. 2007;25:3376-84 pubmed
    ..These results indicate that reelin signaling plays a pivotal role in the dopaminergic system in adult mice, especially in postsynaptic levels. ..
  26. Cabraja M, Bäurle J. Vestibular ganglion neurons survive hair cell defects in jerker, shaker, and Varitint-waddler mutants and downregulate calretinin expression. J Comp Neurol. 2007;504:418-26 pubmed
    ..Whether the decrease in Calr expression may reflect biochemical adaptations in response to input disturbances or a specific loss of large BNs is discussed. ..
  27. K B, V T, N P, M M, N M, F A. Increased sensitivity in the interaction of the dopaminergic/adenosinergic system at the level of the adenylate cyclase activity in the striatum of the "weaver" mouse. Neurochem Int. 2016;99:233-238 pubmed publisher
  28. Anderson C, Crimmins S, Wilson J, Korbel G, Ploegh H, Wilson S. Loss of Usp14 results in reduced levels of ubiquitin in ataxia mice. J Neurochem. 2005;95:724-31 pubmed
    ..These results indicate that Usp14 functions to maintain the cellular levels of monomeric ubiquitin in mammalian cells, and that alterations in the levels of ubiquitin may contribute to neurological disease. ..
  29. Meyer M, Gonzalez Deniselle M, Garay L, Monachelli G, Lima A, Roig P, et al. Stage dependent effects of progesterone on motoneurons and glial cells of wobbler mouse spinal cord degeneration. Cell Mol Neurobiol. 2010;30:123-35 pubmed publisher
  30. Leussis M, Frayne M, Saito M, Berry E, Aldinger K, Rockwell G, et al. Genomic survey of prepulse inhibition in mouse chromosome substitution strains. Genes Brain Behav. 2009;8:806-16 pubmed publisher
    ..Identification of genes that modulate PPI will provide insight into the neural mechanisms underlying sensorimotor gating, as well as the psychopathology of disorders characterized by gating deficits. ..
  31. Costa A, Scott McKean J, Stasko M. Acute injections of the NMDA receptor antagonist memantine rescue performance deficits of the Ts65Dn mouse model of Down syndrome on a fear conditioning test. Neuropsychopharmacology. 2008;33:1624-32 pubmed
    ..These results are very promising from a potential therapeutic perspective, given memantine's current status as a Food and Drug Administration (FDA)-approved drug. ..
  32. Torban E, Patenaude A, Leclerc S, Rakowiecki S, Gauthier S, Andelfinger G, et al. Genetic interaction between members of the Vangl family causes neural tube defects in mice. Proc Natl Acad Sci U S A. 2008;105:3449-54 pubmed publisher
  33. Armstrong C, Chung S, Armstrong J, Hochgeschwender U, Jeong Y, Hawkes R. A novel somatostatin-immunoreactive mossy fiber pathway associated with HSP25-immunoreactive purkinje cell stripes in the mouse cerebellum. J Comp Neurol. 2009;517:524-38 pubmed publisher
    ..Likewise, the Purkinje cell patterning antigens zebrin II and HSP25 are expressed normally in both Sst- and Sst-receptor knockout mice, suggesting that somatostatinergic transmission is not necessary for Purkinje cell stripe formation. ..
  34. Etheredge J, Murchison D, Abbott L, Griffith W. Functional compensation by other voltage-gated Ca2+ channels in mouse basal forebrain neurons with Ca(V)2.1 mutations. Brain Res. 2007;1140:105-19 pubmed
  35. Toro C, Deakin J. Adult neurogenesis and schizophrenia: a window on abnormal early brain development?. Schizophr Res. 2007;90:1-14 pubmed
    ..This is particularly relevant for brain disorders with developmental origins such as schizophrenia. ..
  36. HOE H, Lee K, Carney R, Lee J, Markova A, Lee J, et al. Interaction of reelin with amyloid precursor protein promotes neurite outgrowth. J Neurosci. 2009;29:7459-73 pubmed publisher
    ..Addition of an alpha3beta1 integrin antibody prevented APP and Reelin-induced neurite outgrowth. These findings demonstrate that Reelin interacts with APP, potentially having important effects on neurite development. ..
  37. Merte J, Jensen D, Wright K, Sarsfield S, Wang Y, Schekman R, et al. Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closure. Nat Cell Biol. 2010;12:41-6; sup pp 1-8 pubmed publisher
    ..Thus, during COPII vesicle formation, Sec24b shows cargo specificity for a core PCP component, Vangl2, of which proper ER-to-Golgi transport is essential for the establishment of PCP, convergent extension and closure of the neural tube. ..
  38. Guastavino J, Boufares S, Crusio W. Ovarian abnormalities in the staggerer mutant mouse. ScientificWorldJournal. 2005;5:661-4 pubmed
    ..These results indicate a pleiotropic effect on fertility of the Rora(sg) gene underlying the cerebellar abnormalities of the staggerer mutant. ..
  39. Ferrere A, Vitalis T, Gingras H, Gaspar P, Cases O. Expression of Cux-1 and Cux-2 in the developing somatosensory cortex of normal and barrel-defective mice. Anat Rec A Discov Mol Cell Evol Biol. 2006;288:158-65 pubmed
    ..Our results indicate that Cux-1 is a useful marker of the layer IV neurons in S1, and that Cux-1 and Cux-2 are differently regulated in the upper layers of the cerebral cortex. ..
  40. Kobayashi S, Ito A, Okuzaki D, Onda H, Yabuta N, Nagamori I, et al. Expression profiling of PBMC-based diagnostic gene markers isolated from vasculitis patients. DNA Res. 2008;15:253-65 pubmed publisher
    ..Thus, our large-scale gene profiling study may help finding sensitive and specific DNA markers for diagnosing autoimmune diseases including vasculitis and SLE. ..
  41. Li X, Sapp E, Chase K, Comer Tierney L, Masso N, Alexander J, et al. Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease. Neurobiol Dis. 2009;36:374-83 pubmed publisher
    ..Partial rescue from glutamate-induced cell death occurred in HD neurons expressing dominant active Rab11. We propose a novel mechanism of HD pathogenesis arising from diminished Rab11 activity at recycling endosomes. ..
  42. Miyazaki T, Hashimoto K, Uda A, Sakagami H, Nakamura Y, Saito S, et al. Disturbance of cerebellar synaptic maturation in mutant mice lacking BSRPs, a novel brain-specific receptor-like protein family. FEBS Lett. 2006;580:4057-64 pubmed
    ..Because cerebellar maturation and plasticity require metabotropic glutamate receptor signaling and resulting PKC activation, BSRPs are likely involved in ER functions supporting PKCalpha activation in PCs. ..
  43. Chung W, Kim K, Cho Y, Cho D, Woo J, Ryoo Z, et al. Cochlear pathology of the circling mouse: a new mouse model of DFNB6. Acta Otolaryngol. 2007;127:244-51 pubmed
    ..The number of spiral ganglion neurons significantly reduced with age. RT-PCR analysis indicated that the transmembrane inner ear gene (Tmie) was absent in various organs in circling mice. ..
  44. Yaguchi T, Nagata T, Mukasa T, Fujikawa H, Yamamoto H, Yamamoto S, et al. Linoleic acid derivative DCP-LA improves learning impairment in SAMP8. Neuroreport. 2006;17:105-8 pubmed
    ..The results of the present study, thus, suggest that DCP-LA could improve age-related learning impairment by enhancing cognitive functions. ..
  45. Han Y, Spassky N, Romaguera Ros M, Garcia Verdugo J, Aguilar A, Schneider Maunoury S, et al. Hedgehog signaling and primary cilia are required for the formation of adult neural stem cells. Nat Neurosci. 2008;11:277-84 pubmed publisher
    ..We conclude that Shh signaling, acting through the primary cilia, has a critical role in the expansion and establishment of postnatal hippocampal progenitors. ..
  46. Schwander M, Lopes V, Sczaniecka A, Gibbs D, Lillo C, Delano D, et al. A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells. J Neurosci. 2009;29:15810-8 pubmed publisher
    ..Our findings also suggest that MYO7A mutations can lead to tissue-specific effects on protein levels, which may explain why some mutations in MYO7A lead to deafness without retinal impairment. ..
  47. Kitaura J, Kawakami Y, Maeda Yamamoto M, Horejsi V, Kawakami T. Dysregulation of Src family kinases in mast cells from epilepsy-resistant ASK versus epilepsy-prone EL mice. J Immunol. 2007;178:455-62 pubmed
  48. Herrmann P, Druckrey Fiskaaen C, Kouznetsova E, Heinitz K, Bigl M, Cotman S, et al. Developmental impairments of select neurotransmitter systems in brains of Cln3(Deltaex7/8) knock-in mice, an animal model of juvenile neuronal ceroid lipofuscinosis. J Neurosci Res. 2008;86:1857-70 pubmed publisher
    ..Detailed elucidation of the sequence of pathological events during postnatal development highlights new potential strategies for symptomatic treatment of the disease. ..
  49. Yavich L, Oksman M, Tanila H, Kerokoski P, Hiltunen M, van Groen T, et al. Locomotor activity and evoked dopamine release are reduced in mice overexpressing A30P-mutated human alpha-synuclein. Neurobiol Dis. 2005;20:303-13 pubmed
    ..This is in agreement with the previously postulated involvement of alpha-synuclein in the turnover of transmitter vesicles and may explain the observed motor deficits in A30P mice. ..
  50. Vrijens K, Thys S, De Jeu M, Postnov A, Pfister M, Cox L, et al. Ozzy, a Jag1 vestibular mouse mutant, displays characteristics of Alagille syndrome. Neurobiol Dis. 2006;24:28-40 pubmed
    ..Thirty-seven percent of Ozzy mice showed heart defects. No eye or vertebral abnormalities could be detected. In conclusion, Ozzy mice show two of the major and one minor characteristic of AGS...
  51. Reiserer R, Harrison F, Syverud D, McDonald M. Impaired spatial learning in the APPSwe + PSEN1DeltaE9 bigenic mouse model of Alzheimer's disease. Genes Brain Behav. 2007;6:54-65 pubmed
    ..Seven-month-old APP(Swe) + PSEN1DeltaE9 mice were unimpaired on tests of memory that did not involve learning the rules governing spatial associations...
  52. Lee N, Jeong Y. Pogo: a novel spontaneous ataxic mutant mouse. Cerebellum. 2009;8:155-62 pubmed publisher
    ..Much of this review is concerned with the functional implications of these ectopic molecules in the Pogo cerebellum...
  53. Sachs A, David S, Haider N, Nystuen A. Patterned neuroprotection in the Inpp4a(wbl) mutant mouse cerebellum correlates with the expression of Eaat4. PLoS ONE. 2009;4:e8270 pubmed publisher
    ..Our data suggest that Purkinje cell loss in the Inpp4a(wbl) mutant is due to glutamate excitotoxicity initiated by the climbing fiber, and that Eaat4 may exert a protective effect...
  54. Shibuya S, Wakayama Y, Inoue M, Kojima H, Oniki H. Merosin (laminin-2) localization in basal lamina of normal skeletal muscle fibers and changes in plasma membrane of merosin-deficient skeletal muscle fibers. Med Electron Microsc. 2003;36:213-20 pubmed
    ..Thus, further studies including the functional role of OAs would be required to understand the pathomechanism of merosin-deficient CMD...
  55. Jeong Y, Chung S, Kim C, Kim K, Han S, Hyun B, et al. Corticotropin-releasing factor immunoreactivity increases in the cerebellar climbing fibers in the novel ataxic mutant mouse, pogo. Anat Histol Embryol. 2006;35:111-5 pubmed
    ..Therefore, we suggest that an increase of CRF level may alter the function of targeted Purkinje cells and that it is related to the ataxic phenotype in the pogo mutant mouse...
  56. Martin M, Hiltner T, Wood J, Fraser S, Jacobs R, Readhead C. Myelin deficiencies visualized in vivo: visually evoked potentials and T2-weighted magnetic resonance images of shiverer mutant and wild-type mice. J Neurosci Res. 2006;84:1716-26 pubmed
    ..These data show the potential of using micro MRI in combination with VEPs to follow changes in both the quality and the quantity of myelin in vivo. These combined methods would be useful for longitudinal studies and therapy testing...
  57. McFarland R, Blokhin A, Sydnor J, Mariani J, Vogel M. Oxidative stress, nitric oxide, and the mechanisms of cell death in Lurcher Purkinje cells. Dev Neurobiol. 2007;67:1032-46 pubmed
  58. Keays D. Neuronal migration: unraveling the molecular pathway with humans, mice, and a fungus. Mamm Genome. 2007;18:425-30 pubmed
  59. Parkhouse W, Cunningham L, McFee I, Miller J, Whitney D, Pelech S, et al. Neuromuscular dysfunction in the mutant superoxide dismutase mouse model of amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008;9:24-34 pubmed publisher
    ..These data suggest that the debility in the mSOD mouse model of ALS is produced, in part, by impaired retrograde uptake/transport in motor neuron axons in spite of regenerative support from muscle such as elevated expression of PKB...
  60. Akopians A, Babayan A, Beffert U, Herz J, Basbaum A, Phelps P. Contribution of the Reelin signaling pathways to nociceptive processing. Eur J Neurosci. 2008;27:523-37 pubmed publisher
  61. MacPherson P, McGaffigan R, Wahlsten D, Nguyen P. Impaired fear memory, altered object memory and modified hippocampal synaptic plasticity in split-brain mice. Brain Res. 2008;1210:179-88 pubmed publisher
    ..Agenesis of the CC and HC may unmask some latent ability to encode, store or retrieve certain forms of recognition memory. We suggest that the increased susceptibility to DPT in BTBR may underlie the memory phenotype reported here...
  62. Swinny J, van der Want J, Gramsbergen A. Cerebellar development and plasticity: perspectives for motor coordination strategies, for motor skills, and for therapy. Neural Plast. 2005;12:153-60; discussion 263-72 pubmed
    ..This understanding will also help in the approach to possible therapies of pathologies arising during development because the cerebellum is especially prone to such perturbation because of its late development...
  63. Dubroff J, Stevens R, Hitt J, Hodge C, McCasland J. Anomalous functional organization of barrel cortex in GAP-43 deficient mice. Neuroimage. 2006;29:1040-8 pubmed
    ..Since histological HZ barrels recover normal dimensions by postnatal day 26, the altered circuit function in GAP-43 HZ cortex could be a secondary consequence of the rescue of barrel dimensions...
  64. Holland K, Fleming M, Cheek S, Moran J, Beier D, Meisler M. De novo exon duplication in a new allele of mouse Glra1 (spasmodic). Genetics. 2006;174:2245-7 pubmed
    ..The mutant transcript results in premature protein truncation. A direct repeat of the pentamer GGGGC is present adjacent to the breakpoints and may have mediated the duplication event by a replication slippage mechanism...
  65. Ognibene E, Adriani W, Caprioli A, Ghirardi O, Ali S, Aloe L, et al. The effect of early maternal separation on brain derived neurotrophic factor and monoamine levels in adult heterozygous reeler mice. Prog Neuropsychopharmacol Biol Psychiatry. 2008;32:1269-76 pubmed publisher
    ..The modulatory effects of a chronic treatment with the atypical antipsychotic olanzapine (OLZ, 1.5 mg/kg for 40 days) were also investigated...
  66. Repici M, Zanjani H, Gautheron V, Borsello T, Dusart I, Mariani J. Specific JNK inhibition by D-JNKI1 protects Purkinje cells from cell death in Lurcher mutant mouse. Cerebellum. 2008;7:534-8 pubmed publisher
    ..Conversely, this specific JNK inhibitor cell permeable peptide did not increase PC number in +/+ treated versus untreated cultures. These results clearly indicate that JNK plays an important role in +/Lc PC mechanism of cell death...
  67. Katsuyama Y, Terashima T. Developmental anatomy of reeler mutant mouse. Dev Growth Differ. 2009;51:271-86 pubmed publisher
    ..Based on this overview of the reeler abnormalities, we discuss possible function of Reelin signal in the neuronal migration and other morphological events in mouse development...
  68. Gilerovich E, Grigor ev I. Gaba- and serotonin-immunoreactive structures and ca(2+)-binding protein in the neocortex of the reeler mouse mutant. Neurosci Behav Physiol. 2005;35:887-90 pubmed
    ..Thus, the anomalously formed neocortex which lacks the typical cytoarchitectonic organization, showed abnormalities in the structure of both intrinsic and projectional transmitter systems...
  69. Fukazawa N, Ayukawa K, Nishikawa K, Ohashi H, Ichihara N, Hikawa Y, et al. Identification and functional characterization of mouse TPO1 as a myelin membrane protein. Brain Res. 2006;1070:1-14 pubmed
    ..Furthermore, TPO1 selectively colocalized with non-phosphorylated Fyn and promoted Fyn autophosphorylation in COS7 cells, suggesting that TPO1 may play a role in myelin formation via Fyn kinase activation in the PNS and CNS...
  70. Dickey C, Yue M, Lin W, Dickson D, Dunmore J, Lee W, et al. Deletion of the ubiquitin ligase CHIP leads to the accumulation, but not the aggregation, of both endogenous phospho- and caspase-3-cleaved tau species. J Neurosci. 2006;26:6985-96 pubmed
    ..Based on these findings, we propose that polyubiquitination of tau by CHIP may facilitate the formation of insoluble filamentous tau lesions...
  71. Forcelli P, Orefice L, Heinrichs S. Neural, endocrine and electroencephalographic hyperreactivity to human contact: a diathesis-stress model of seizure susceptibility in El mice. Brain Res. 2007;1144:248-56 pubmed
    ..The findings support a diathesis-stress hypothesis in which genetically seizure susceptible El mice exhibit a multifaceted hyperreactivity to noxious environmental stimuli...
  72. Marti J, Santa Cruz M, Bayer S, Ghetti B, Hervas J. Purkinje cell age-distribution in fissures and in foliar crowns: a comparative study in the weaver cerebellum. Brain Struct Funct. 2007;212:347-57 pubmed
  73. Menuz K, Nicoll R. Loss of inhibitory neuron AMPA receptors contributes to ataxia and epilepsy in stargazer mice. J Neurosci. 2008;28:10599-603 pubmed publisher
  74. Buran B, Strenzke N, Neef A, Gundelfinger E, Moser T, Liberman M. Onset coding is degraded in auditory nerve fibers from mutant mice lacking synaptic ribbons. J Neurosci. 2010;30:7587-97 pubmed publisher
  75. Fang C, Bernardes Silva M, Coleman M, Perry V. The cellular distribution of the Wld s chimeric protein and its constituent proteins in the CNS. Neuroscience. 2005;135:1107-18 pubmed
    ..The increased Nmnat-1 activity in the nucleus of Wld s mice compared with wild-type mice seems to be a significant factor in the axon protection. It is not known whether the expression of the Nmnat-1 in the axon is significant...
  76. Wolf R, Dobrowolny H, Matzke K, Paelchen K, Bogerts B, Schwegler H. Prepulse inhibition is different in two inbred mouse strains (CPB-K and BALB/cJ) with different hippocampal NMDA receptor densities. Behav Brain Res. 2006;166:78-84 pubmed
    ..For this purpose, the inbred mouse strains CPB-K and BALB/cJ were compared by using a sensorimotor gating paradigm...
  77. Fureman B, Hess E. Noradrenergic blockade prevents attacks in a model of episodic dysfunction caused by a channelopathy. Neurobiol Dis. 2005;20:227-32 pubmed
    ..These results suggest that, while noradrenergic neurotransmission may be necessary for attacks, an increase in norepinephrine is not sufficient to induce attacks...
  78. Song J, Goetz B, Duncan I. His36Pro point-mutated proteolipid protein retained in the endoplasmic reticulum of oligodendrocytes in the shaking pup. Glia. 2006;53:257-65 pubmed
    ..These results suggest that mutated PLP/DM20 in the shp could be selectively retained in RER, causing disruption of their translocation to the periphery to myelinate axons...
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    ..This work contributes to our understanding of the adaptive changes occurring in response to a lack of myelin in a noninflammatory environment similar to the situation seen in chronically demyelinated MS plaques...
  80. McKay S, Oleskevich S. The role of spontaneous activity in development of the endbulb of Held synapse. Hear Res. 2007;230:53-63 pubmed
    ..7-fold) after hearing onset while release probability remained constant (0.5). Overall, the results in deafness mice suggest that synaptic strength is altered in the absence of spontaneous auditory nerve activity...
  81. Janmaat S, Frederic F, Sjollema K, Luiten P, Mariani J, van der Want J. Formation and maturation of parallel fiber-Purkinje cell synapses in the Staggerer cerebellum ex vivo. J Comp Neurol. 2009;512:467-77 pubmed publisher
    ..The presynaptic distribution of VGluT1 and VGluT2 on Rora(sg/sg) PCs matched the postsynaptic distribution of the glutamate receptor GluRdelta2, but not mGluR1...
  82. Petzold A, Stiefel D, Copp A. Amniotic fluid brain-specific proteins are biomarkers for spinal cord injury in experimental myelomeningocele. J Neurochem. 2005;95:594-8 pubmed
    ..5 and E18.5, suggesting that axonal degeneration is most severe in large MMC. Amniotic fluid BSP measurements may provide important information for balancing the risks and benefits to mother and child of in utero surgery for MMC...
  83. Isosaka T, Hattori K, Yagi T. NMDA-receptor proteins are upregulated in the hippocampus of postnatal heterozygous reeler mice. Brain Res. 2006;1073-1074:11-9 pubmed
    ..Thus, the NMDA-R protein level is regulated by the Reelin protein level in a Fyn-dependent manner in the mouse brain...
  84. Gheorghita F, Kraftsik R, Dubois R, Welker E. Structural basis for map formation in the thalamocortical pathway of the barrelless mouse. J Neurosci. 2006;26:10057-67 pubmed
    ..We propose that the domains of high bouton density are at the basis of the whisker map in barrelless mice...
  85. Jossin Y, Gui L, Goffinet A. Processing of Reelin by embryonic neurons is important for function in tissue but not in dissociated cultured neurons. J Neurosci. 2007;27:4243-52 pubmed
  86. Derenne A, Arsenault M, Austin D, Weatherly J. Weaver mutant mice exhibit long-term learning deficits under several measures of instrumental behavior. Physiol Behav. 2007;92:1002-9 pubmed
    ..The findings suggest that weaver mutant mice express learning deficits similar to those found in other dopamine-deficient organisms...
  87. Bando Y, Takakusaki K, Ito S, Terayama R, Kashiwayanagi M, Yoshida S. Differential changes in axonal conduction following CNS demyelination in two mouse models. Eur J Neurosci. 2008;28:1731-42 pubmed publisher
  88. Virard I, Coquillat D, Bancila M, Kaing S, Durbec P. Oligodendrocyte precursor cells generate pituicytes in vivo during neurohypophysis development. Glia. 2006;53:294-303 pubmed
    ..This constitutes the first demonstration that OPCs generate glial cells other than oligodendrocytes in vivo...
  89. Linnemann C, Schmeh I, Thier P, Schwarz C. Transient change in GABA(A) receptor subunit mRNA expression in Lurcher cerebellar nuclei during Purkinje cell degeneration. BMC Neurosci. 2006;7:59 pubmed
    ..Here, we further investigated this phenomenon, using real-time RT-PCR to assess GABAA receptor subunit gene expression during PC degeneration...
  90. Litwack E, Lee Y, Mallott J. Absence of the basilar pons in mice lacking a functional Large glycosyltransferase gene suggests a defect in pontine neuron migration. Brain Res. 2006;1117:12-7 pubmed
    ..In addition, the basilar pons forms normally in dystrophin-deficient mice. These results demonstrate that the Large glycosyltransferase but not dystrophin is required for normal basilar pontine development...
  91. Shelbourne P, Coote E, Dadak S, Cobb S. Normal electrical properties of hippocampal neurons modelling early Huntington disease pathogenesis. Brain Res. 2007;1139:226-34 pubmed
    ..Furthermore, together with our previous work, these findings point to a synaptic rather than cellular locus of HD-related pathology...
  92. Pascual J, Heinrichs S. Olfactory neophobia and seizure susceptibility phenotypes in an animal model of epilepsy are normalized by impairment of brain corticotropin releasing factor. Epilepsia. 2007;48:827-33 pubmed
    ..The present study explored the causal relationship between stressor exposure/stress neuropeptide activation and avoidant exploratory phenotype/enhanced seizure susceptibility in an animal model of epilepsy...