Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease
about
Anti-inflammatory therapy by ibudilast, a phosphodiesterase inhibitor, in demyelination of twitcher, a genetic demyelination modelInsights into Krabbe disease from structures of galactocerebrosidaseNeuroimmune mechanisms in Krabbe's diseaseMolecular beacon genotyping for globoid cell leukodystrophy from hair roots in the twitcher mouse and rhesus macaqueTherapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophySize and specimen-dependent strategy for x-ray micro-ct and tem correlative analysis of nervous system samplesMulti-system disorders of glycosphingolipid and ganglioside metabolismDiffusion tensor imaging detects axonal injury and demyelination in the spinal cord and cranial nerves of a murine model of globoid cell leukodystrophy.Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins.Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in miceEffect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease.Persistence of psychosine in brain lipid rafts is a limiting factor in the therapeutic recovery of a mouse model for Krabbe disease.Central nervous system pathology progresses independently of KC and CXCR2 in globoid-cell leukodystrophyMacrophages counteract demyelination in a mouse model of globoid cell leukodystrophyEarly axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease.Mechanism of neuromuscular dysfunction in Krabbe disease.The galactocerebrosidase enzyme contributes to the maintenance of a functional hematopoietic stem cell niche.Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophyThe myelin mutants as models to study myelin repair in the leukodystrophies.Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy.Design of a regulated lentiviral vector for hematopoietic stem cell gene therapy of globoid cell leukodystrophyBone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophyProgress in multiple sclerosis genetics.Long-term Improvements in Lifespan and Pathology in CNS and PNS After BMT Plus One Intravenous Injection of AAVrh10-GALC in Twitcher MiceThe sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors.Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe diseaseInhibition of angiogenesis by β-galactosylceramidase deficiency in globoid cell leukodystrophy.Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy.Mouse forward genetics in the study of the peripheral nervous system and human peripheral neuropathyA microglial hypothesis of globoid cell leukodystrophy pathology.Metabolic profiling reveals biochemical pathways and potential biomarkers associated with the pathogenesis of Krabbe disease.Reversibility of neuropathology in Tay-Sachs-related diseases.Characterization and application of a disease-cell model for a neurodegenerative lysosomal disease.Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.Advances and pitfalls of cell therapy in metabolic leukodystrophies.Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease.Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants.Myelin repair by transplantation of myelin-forming cells in globoid cell leukodystrophy.Krabbe disease: One Hundred years from the bedside to the bench to the bedside.Treatment for Krabbe's disease: Finding the combination.
P2860
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P2860
Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease
description
1996 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի մարտին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 1996
@ast
scientific journal article
@en
vedecký článok (publikovaný 1996/03/01)
@sk
vědecký článek publikovaný v roce 1996
@cs
wetenschappelijk artikel (gepubliceerd op 1996/03/01)
@nl
наукова стаття, опублікована в березні 1996
@uk
مقالة علمية (نشرت في مارس 1996)
@ar
name
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@ast
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@en
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@nl
type
label
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@ast
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@en
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@nl
prefLabel
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@ast
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@en
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@nl
P2093
P1476
Molecular cloning and expressi ...... e, a model of Krabbe's disease
@en
P2093
H. Fukushima
H. Tsukamoto
I. Yanagihara
J. Nishimoto
M. Taniike
N. Tatsumi
P304
P356
10.1046/J.1471-4159.1996.66031118.X
P407
P577
1996-03-01T00:00:00Z