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Search results 1 to 100 out of 184 for Galc

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Category: Publication
Type Details Score
Publication      
First Author: Karst SY
Year: 2008
Journal: MGI Direct Data Submission
Title: Twitcher 5 Jackson; a new remutation in the Galc gene
Publication
First Author: Weinstock NI
Year: 2020
Journal: Neuron
Title: Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction.
Volume: 107
Issue: 1
Pages: 65-81.e9
Publication
First Author: Victoria T
Year: 1996
Journal: Genomics
Title: Cloning of the canine GALC cDNA and identification of the mutation causing globoid cell leukodystrophy in West Highland White and Cairn terriers.
Volume: 33
Issue: 3
Pages: 457-62
Publication
First Author: Potter GB
Year: 2013
Journal: Hum Mol Genet
Title: Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe disease.
Volume: 22
Issue: 17
Pages: 3397-414
Publication      
First Author: Schroeder D
Year: 2008
Journal: MGI Direct Data Submission
Title: Twitcher 4 Jackson, an ENU induced mutation in Galc
Publication      
First Author: Prince L
Year: 2018
Journal: MGI Direct Data Submission
Title: Mutagenetix entry for teeter.
Publication      
First Author: Prince L
Year: 2019
Journal: MGI Direct Data Submission
Title: Mutagenetix entry for quake.
Publication
First Author: Zlotogora J
Year: 1990
Journal: Am J Hum Genet
Title: Krabbe disease locus mapped to chromosome 14 by genetic linkage.
Volume: 47
Issue: 1
Pages: 37-44
Publication
First Author: Pellegatta S
Year: 2006
Journal: Neurobiol Dis
Title: The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation.
Volume: 21
Issue: 2
Pages: 314-23
Publication
First Author: Visigalli I
Year: 2010
Journal: Blood
Title: The galactocerebrosidase enzyme contributes to the maintenance of a functional hematopoietic stem cell niche.
Volume: 116
Issue: 11
Pages: 1857-66
Publication
First Author: Mikulka CR
Year: 2020
Journal: Proc Natl Acad Sci U S A
Title: Cell-autonomous expression of the acid hydrolase galactocerebrosidase.
Volume: 117
Issue: 16
Pages: 9032-9041
Publication
First Author: Deane JE
Year: 2011
Journal: Proc Natl Acad Sci U S A
Title: Insights into Krabbe disease from structures of galactocerebrosidase.
Volume: 108
Issue: 37
Pages: 15169-73
Publication  
First Author: Rebiai R
Year: 2022
Journal: Front Mol Neurosci
Title: CRISPR-Cas9 Knock-In of T513M and G41S Mutations in the Murine β-Galactosyl-Ceramidase Gene Re-capitulates Early-Onset and Adult-Onset Forms of Krabbe Disease.
Volume: 15
Pages: 896314
Publication  
First Author: Luzi P
Year: 2009
Journal: Brain Res
Title: Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy.
Volume: 1300
Pages: 146-58
Publication
First Author: Ricca A
Year: 2015
Journal: Hum Mol Genet
Title: Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy.
Volume: 24
Issue: 12
Pages: 3372-89
Publication
First Author: Scott-Hewitt NJ
Year: 2017
Journal: Hum Mol Genet
Title: Heterozygote galactocerebrosidase (GALC) mutants have reduced remyelination and impaired myelin debris clearance following demyelinating injury.
Volume: 26
Issue: 15
Pages: 2825-2837
Publication  
First Author: Inamura N
Year: 2018
Journal: Neurobiol Dis
Title: Developmental defects and aberrant accumulation of endogenous psychosine in oligodendrocytes in a murine model of Krabbe disease.
Volume: 120
Pages: 51-62
Publication  
First Author: Alabed HBR
Year: 2023
Journal: Biomolecules
Title: Untargeted Lipidomic Approach for Studying Different Nervous System Tissues of the Murine Model of Krabbe Disease.
Volume: 13
Issue: 10
Publication
First Author: Shen JS
Year: 2001
Journal: Gene Ther
Title: Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements.
Volume: 8
Issue: 14
Pages: 1081-7
Publication
First Author: Paintlia MK
Year: 2015
Journal: J Neurosci Res
Title: Effect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease.
Volume: 93
Issue: 1
Pages: 28-42
Publication
First Author: Santambrogio S
Year: 2012
Journal: Hum Mol Genet
Title: The galactocerebrosidase enzyme contributes to maintain a functional neurogenic niche during early post-natal CNS development.
Volume: 21
Issue: 21
Pages: 4732-50
Publication
First Author: Costantino-Ceccarini E
Year: 1999
Journal: Neurochem Res
Title: Transduction of cultured oligodendrocytes from normal and twitcher mice by a retroviral vector containing human galactocerebrosidase (GALC) cDNA.
Volume: 24
Issue: 2
Pages: 287-93
Publication
First Author: Luddi A
Year: 2005
Journal: Exp Cell Res
Title: Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy.
Volume: 304
Issue: 1
Pages: 59-68
Publication
First Author: Qin EY
Year: 2012
Journal: Mol Genet Metab
Title: Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy.
Volume: 107
Issue: 1-2
Pages: 186-96
Publication
First Author: Hawkins-Salsbury JA
Year: 2015
Journal: J Neurosci
Title: Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy.
Volume: 35
Issue: 16
Pages: 6495-505
Publication
First Author: White AB
Year: 2009
Journal: J Neurosci
Title: Psychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture.
Volume: 29
Issue: 19
Pages: 6068-77
Publication
First Author: Weinstock NI
Year: 2020
Journal: Nat Commun
Title: Brainstem development requires galactosylceramidase and is critical for pathogenesis in a model of Krabbe disease.
Volume: 11
Issue: 1
Pages: 5356
Publication  
First Author: Zaccariotto E
Year: 2022
Journal: Biomed Pharmacother
Title: A novel brain-penetrant oral UGT8 inhibitor decreases in vivo galactosphingolipid biosynthesis in murine Krabbe disease.
Volume: 149
Pages: 112808
Publication
First Author: Luzi P
Year: 2001
Journal: Mol Genet Metab
Title: Generation of a mouse with low galactocerebrosidase activity by gene targeting: a new model of globoid cell leukodystrophy (Krabbe disease).
Volume: 73
Issue: 3
Pages: 211-23
Publication
First Author: Luzi P
Year: 2005
Journal: Mol Genet Metab
Title: Biochemical and pathological evaluation of long-lived mice with globoid cell leukodystrophy after bone marrow transplantation.
Volume: 86
Issue: 1-2
Pages: 150-9
Publication
First Author: Béchet S
Year: 2020
Journal: J Neurosci
Title: Fingolimod Rescues Demyelination in a Mouse Model of Krabbe's Disease.
Volume: 40
Issue: 15
Pages: 3104-3118
Publication
First Author: Lin DS
Year: 2015
Journal: Gene
Title: Mitigation of cerebellar neuropathy in globoid cell leukodystrophy mice by AAV-mediated gene therapy.
Volume: 571
Issue: 1
Pages: 81-90
Publication
First Author: Weinstock NI
Year: 2016
Journal: J Neurosci Res
Title: Metabolic profiling reveals biochemical pathways and potential biomarkers associated with the pathogenesis of Krabbe disease.
Volume: 94
Issue: 11
Pages: 1094-107
Publication
First Author: Hu P
Year: 2016
Journal: J Neurosci Res
Title: Hematopoietic Stem cell transplantation and lentiviral vector-based gene therapy for Krabbe's disease: Present convictions and future prospects.
Volume: 94
Issue: 11
Pages: 1152-68
Publication
First Author: Rafi MA
Year: 2015
Journal: Mol Genet Metab
Title: Intravenous injection of AAVrh10-GALC after the neonatal period in twitcher mice results in significant expression in the central and peripheral nervous systems and improvement of clinical features.
Volume: 114
Issue: 3
Pages: 459-66
Publication
First Author: Rafi MA
Year: 2021
Journal: Bioimpacts
Title: Can early treatment of twitcher mice with high dose AAVrh10-GALC eliminate the need for BMT?
Volume: 11
Issue: 2
Pages: 135-146
Publication
First Author: Taylor RM
Year: 2006
Journal: J Neurochem
Title: Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophy.
Volume: 97
Issue: 6
Pages: 1585-99
Publication
First Author: Taniike M
Year: 2002
Journal: J Neurosci
Title: Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model.
Volume: 22
Issue: 12
Pages: 4885-96
Publication
First Author: Ezoe T
Year: 2000
Journal: J Neurosci Res
Title: Twitcher mice with only a single active galactosylceramide synthase gene exhibit clearly detectable but therapeutically minor phenotypic improvements.
Volume: 59
Issue: 2
Pages: 179-87
Publication
First Author: Ezoe T
Year: 2000
Journal: J Neurosci Res
Title: Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide.
Volume: 59
Issue: 2
Pages: 170-8
Publication
First Author: Ijichi K
Year: 2013
Journal: Glia
Title: MMP-3 mediates psychosine-induced globoid cell formation: implications for leukodystrophy pathology.
Volume: 61
Issue: 5
Pages: 765-77
Publication
First Author: Reddy AS
Year: 2014
Journal: PLoS One
Title: Central nervous system pathology progresses independently of KC and CXCR2 in globoid-cell leukodystrophy.
Volume: 8
Issue: 6
Pages: e64647
Publication
First Author: Matsuda J
Year: 2001
Journal: Hum Mol Genet
Title: A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse.
Volume: 10
Issue: 11
Pages: 1191-9
Publication
First Author: Sakai N
Year: 1996
Journal: J Neurochem
Title: Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease.
Volume: 66
Issue: 3
Pages: 1118-24
Publication
First Author: Cantuti-Castelvetri L
Year: 2015
Journal: J Neurosci
Title: Mechanism of neuromuscular dysfunction in Krabbe disease.
Volume: 35
Issue: 4
Pages: 1606-16
Publication
First Author: Duchen LW
Year: 1980
Journal: Brain
Title: Hereditary leucodystrophy in the mouse: the new mutant twitcher.
Volume: 103
Issue: 3
Pages: 695-710
Publication
First Author: Skiba MC
Year: 1990
Journal: J Neurosci Res
Title: Somatic cell genetic analysis of the galactocerebrosidase gene: lack of complementation in human Krabbe disease/twitcher mouse cell hybrids.
Volume: 27
Issue: 4
Pages: 472-8
Publication
First Author: Scaravilli F
Year: 1981
Journal: Nature
Title: Peripheral nerve grafts in hereditary leukodystrophic mutant mice (twitcher).
Volume: 290
Issue: 5801
Pages: 56-8
Publication
First Author: Haq E
Year: 2006
Journal: Biochem Biophys Res Commun
Title: Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease.
Volume: 343
Issue: 1
Pages: 229-38
Publication
First Author: Suzuki K
Year: 1995
Journal: Brain Pathol
Title: The twitcher mouse: a model for Krabbe disease and for experimental therapies.
Volume: 5
Issue: 3
Pages: 249-58
Publication
First Author: Galbiati F
Year: 2007
Journal: J Neurosci
Title: Autonomic denervation of lymphoid organs leads to epigenetic immune atrophy in a mouse model of Krabbe disease.
Volume: 27
Issue: 50
Pages: 13730-8
Publication
First Author: Contreras MA
Year: 2010
Journal: Biochim Biophys Acta
Title: Factors that affect postnatal bone growth retardation in the twitcher murine model of Krabbe disease.
Volume: 1802
Issue: 7-8
Pages: 601-8
Publication
First Author: Contreras MA
Year: 2008
Journal: Arch Biochem Biophys
Title: Psychosine-induced alterations in peroxisomes of twitcher mouse liver.
Volume: 477
Issue: 2
Pages: 211-8
Publication
First Author: Higashi Y
Year: 1992
Journal: J Neuropathol Exp Neurol
Title: The twitcher mouse: immunocytochemical study of Ia expression in macrophages.
Volume: 51
Issue: 1
Pages: 47-57
Publication
First Author: Neri M
Year: 2011
Journal: Stem Cells
Title: Neural stem cell gene therapy ameliorates pathology and function in a mouse model of globoid cell leukodystrophy.
Volume: 29
Issue: 10
Pages: 1559-71
Publication
First Author: Powell HC
Year: 1983
Journal: Lab Invest
Title: Peripheral neuropathy in the Twitcher mutant. A new experimental model of endoneurial edema.
Volume: 49
Issue: 1
Pages: 19-25
Publication
First Author: Folts CJ
Year: 2016
Journal: PLoS Biol
Title: Lysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular Toxicity.
Volume: 14
Issue: 12
Pages: e1002583
Publication  
First Author: Sural-Fehr T
Year: 2019
Journal: Dis Model Mech
Title: Inhibition of the IGF-1-PI3K-Akt-mTORC2 pathway in lipid rafts increases neuronal vulnerability in a genetic lysosomal glycosphingolipidosis.
Volume: 12
Issue: 5
Publication
First Author: Ohno M
Year: 1993
Journal: Brain Res
Title: Proliferation of microglia/macrophages in the demyelinating CNS and PNS of twitcher mouse.
Volume: 602
Issue: 2
Pages: 268-74
Publication
First Author: Cantuti Castelvetri L
Year: 2013
Journal: J Neurosci
Title: The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors.
Volume: 33
Issue: 24
Pages: 10048-56
Publication
First Author: D'Auria L
Year: 2017
Journal: PLoS One
Title: Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.
Volume: 12
Issue: 5
Pages: e0178103
Publication
First Author: LeVine SM
Year: 1994
Journal: Int J Dev Neurosci
Title: Neuropathology of twitcher mice: examination by histochemistry, immunohistochemistry, lectin histochemistry and Fourier transform infrared microspectroscopy.
Volume: 12
Issue: 4
Pages: 275-88
Publication
First Author: Wu YP
Year: 2000
Journal: Am J Pathol
Title: Distribution and characterization of GFP(+) donor hematogenous cells in Twitcher mice after bone marrow transplantation.
Volume: 156
Issue: 6
Pages: 1849-54
Publication
First Author: Huppes W
Year: 1992
Journal: J Neurocytol
Title: Detection of migrated allogeneic oligodendrocytes throughout the central nervous system of the galactocerebrosidase-deficient twitcher mouse.
Volume: 21
Issue: 2
Pages: 129-36
Publication
First Author: Komiyama A
Year: 1992
Journal: Brain Res
Title: Progressive impairment of Schwann cell proliferation in vitro in murine globoid cell leukodystrophy (twitcher).
Volume: 598
Issue: 1-2
Pages: 1-9
Publication
First Author: Matsushima GK
Year: 1994
Journal: Cell
Title: Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy.
Volume: 78
Issue: 4
Pages: 645-56
Publication
First Author: Biswas S
Year: 2002
Journal: Neurobiol Dis
Title: Delayed clinical and pathological signs in twitcher (globoid cell leukodystrophy) mice on a C57BL/6 x CAST/Ei background.
Volume: 10
Issue: 3
Pages: 344-57
Publication
First Author: Taniike M
Year: 1995
Journal: J Neurosci Res
Title: Proliferative capacity of oligodendrocytes in the demyelinating twitcher spinal cord.
Volume: 40
Issue: 3
Pages: 325-32
Publication
First Author: Igisu H
Year: 1984
Journal: Science
Title: Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
Volume: 224
Issue: 4650
Pages: 753-5
Publication  
First Author: Duchen LW
Year: 1981
Journal: Mouse News Lett
Title: A new neurological mutant "twitcher" (twi)
Volume: 61
Pages: 47
Publication
First Author: Hawkins-Salsbury JA
Year: 2012
Journal: Exp Neurol
Title: Oxidative stress as a therapeutic target in globoid cell leukodystrophy.
Volume: 237
Issue: 2
Pages: 444-52
Publication
First Author: Taniike M
Year: 1999
Journal: J Neuropathol Exp Neurol
Title: An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy.
Volume: 58
Issue: 6
Pages: 644-53
Publication
First Author: Suzuki K
Year: 1995
Journal: Microsc Res Tech
Title: Murine model of genetic demyelinating disease: the twitcher mouse.
Volume: 32
Issue: 3
Pages: 204-14
Publication
First Author: Scaravilli F
Year: 1983
Journal: Nature
Title: Enzyme replacement in grafted nerve of twitcher mouse.
Volume: 305
Issue: 5936
Pages: 713-5
Publication
First Author: Babcock MC
Year: 2021
Journal: Sci Rep
Title: Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor.
Volume: 11
Issue: 1
Pages: 14486
Publication
First Author: Li Y
Year: 2014
Journal: Pediatr Neurol
Title: Experimental therapies in the murine model of globoid cell leukodystrophy.
Volume: 51
Issue: 5
Pages: 600-6
Publication
First Author: Pannuzzo G
Year: 2010
Journal: Mol Genet Metab
Title: A galactose-free diet enriched in soy isoflavones and antioxidants results in delayed onset of symptoms of Krabbe disease in twitcher mice.
Volume: 100
Issue: 3
Pages: 234-40
Publication  
First Author: Del Grosso A
Year: 2019
Journal: Neurobiol Dis
Title: Dysregulated autophagy as a new aspect of the molecular pathogenesis of Krabbe disease.
Volume: 129
Pages: 195-207
Publication
First Author: Whitfield PD
Year: 2001
Journal: J Lipid Res
Title: Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry.
Volume: 42
Issue: 12
Pages: 2092-5
Publication  
First Author: Lin DS
Year: 2023
Journal: Cells
Title: Rapamycin Alleviates Protein Aggregates, Reduces Neuroinflammation, and Rescues Demyelination in Globoid Cell Leukodystrophy.
Volume: 12
Issue: 7
Publication  
First Author: Tonazzini I
Year: 2020
Journal: Biomolecules
Title: Visual System Impairment in a Mouse Model of Krabbe Disease: The Twitcher Mouse.
Volume: 11
Issue: 1
Publication
First Author: Yeager AM
Year: 1984
Journal: Science
Title: Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse.
Volume: 225
Issue: 4666
Pages: 1052-4
Publication  
First Author: Teixeira CA
Year: 2014
Journal: Neurobiol Dis
Title: Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease.
Volume: 66
Pages: 92-103
Publication
First Author: Yagi T
Year: 2004
Journal: Neurobiol Dis
Title: Fate of donor hematopoietic cells in demyelinating mutant mouse, twitcher, following transplantation of GFP+ bone marrow cells.
Volume: 16
Issue: 1
Pages: 98-109
Publication
First Author: Kobayashi T
Year: 1980
Journal: Brain Res
Title: The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease).
Volume: 202
Issue: 2
Pages: 479-83
Publication
First Author: Igisu H
Year: 1984
Journal: J Neuropathol Exp Neurol
Title: Glycolipids of the spinal cord, sciatic nerve, and systemic organs of the twitcher mouse.
Volume: 43
Issue: 1
Pages: 22-36
Publication
First Author: Takahashi H
Year: 1984
Journal: Lab Invest
Title: Murine globoid cell leukodystrophy: the twitcher mouse. An ultrastructural study of the kidney.
Volume: 50
Issue: 1
Pages: 42-50
Publication
First Author: Taniike M
Year: 1994
Journal: Acta Neuropathol
Title: Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation.
Volume: 88
Issue: 3
Pages: 228-36
Publication      
First Author: Turer E
Year: 2018
Journal: MGI Direct Data Submission
Title: Mutagenetix entry for Crabby2
Publication
First Author: Suzuki K
Year: 1985
Journal: Neurochem Pathol
Title: Genetic galactosylceramidase deficiency (globoid cell leukodystrophy, Krabbe disease) in different mammalian species.
Volume: 3
Issue: 1
Pages: 53-68
Publication
First Author: Ruggeri B
Year: 1991
Journal: Cancer Res
Title: Alterations of the p53 tumor suppressor gene during mouse skin tumor progression.
Volume: 51
Issue: 24
Pages: 6615-21
Publication  
First Author: Suzuki K
Year: 1995
Journal: Prog Brain Res
Title: Expression of immune-related molecules in a murine genetic demyelinating disease.
Volume: 105
Pages: 289-94
Publication
First Author: Kondo Y
Year: 2011
Journal: J Neurosci
Title: Macrophages counteract demyelination in a mouse model of globoid cell leukodystrophy.
Volume: 31
Issue: 10
Pages: 3610-24
Publication
First Author: LeVine SM
Year: 1992
Journal: Brain Res
Title: Morphological features of degenerating oligodendrocytes in twitcher mice.
Volume: 587
Issue: 2
Pages: 348-52
Publication
First Author: Yeager AM
Year: 1993
Journal: Transplantation
Title: Hematopoietic cell transplantation in the twitcher mouse. The effects of pretransplant conditioning with graded doses of busulfan.
Volume: 56
Issue: 1
Pages: 185-90
Publication
First Author: Novikov AM
Year: 1991
Journal: Biochem Genet
Title: Ganglioside GD3 biosynthesis in normal and mutant mouse embryos.
Volume: 29
Issue: 11-12
Pages: 627-38
Publication
First Author: Ono J
Year: 1995
Journal: Brain Res
Title: Differentiation between dysmyelination and demyelination using magnetic resonance diffusional anisotropy.
Volume: 671
Issue: 1
Pages: 141-8
Publication
First Author: Cachón-González MB
Year: 2014
Journal: Hum Mol Genet
Title: Reversibility of neuropathology in Tay-Sachs-related diseases.
Volume: 23
Issue: 3
Pages: 730-48
Publication  
First Author: Coltrini D
Year: 2022
Journal: Int J Mol Sci
Title: β-Galactosylceramidase Deficiency Causes Upregulation of Long Pentraxin-3 in the Central Nervous System of Krabbe Patients and Twitcher Mice.
Volume: 23
Issue: 16
Publication
First Author: Ohno M
Year: 1993
Journal: Brain Res
Title: MHC class II antigen expression and T-cell infiltration in the demyelinating CNS and PNS of the twitcher mouse.
Volume: 625
Issue: 2
Pages: 186-96