about
Intravital assessment of myelin molecular order with polarimetric multiphoton microscopyInsights into Krabbe disease from structures of galactocerebrosidaseStructure of saposin A lipoprotein discsNew therapeutic approaches for Krabbe disease: The potential of pharmacological chaperonesLysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular ToxicityThe role of microglia in brain maintenance: implications for Rett syndrome.Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy.Imaging white matter diffusion changes with development and recovery from brain injury.Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophyOligodendrocyte loss during the disease course in a canine model of the lysosomal storage disease fucosidosisOptic nerve enlargement in Krabbe disease: a pathophysiologic and clinical perspective.Gene therapy for lysosomal storage disorders: a good start.Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.Persistence of psychosine in brain lipid rafts is a limiting factor in the therapeutic recovery of a mouse model for Krabbe disease.Mechanism of neuromuscular dysfunction in Krabbe disease.Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosisProgenitor cell-based treatment of the pediatric myelin disordersDevelopment of mammalian artificial chromosomes for the treatment of genetic diseases: Sandhoff and Krabbe diseases.DNA promoter hypermethylation contributes to down-regulation of galactocerebrosidase gene in lung and head and neck cancersClinical and immunopathologic alterations in rhesus macaques affected with globoid cell leukodystrophyOligodendrocyte death results in immune-mediated CNS demyelination.Krabbe Disease in the Arab World.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.Predominant Corticospinal Tract Involvement in a Late Infant with Krabbe Disease.Large animal models of neurological disorders for gene therapy.Beyond the cherry-red spot: Ocular manifestations of sphingolipid-mediated neurodegenerative and inflammatory disorders.Inherited metabolic disorders involving the eye: a clinico-biochemical perspective.Characterization and application of a disease-cell model for a neurodegenerative lysosomal disease.Patient fibroblasts-derived induced neurons demonstrate autonomous neuronal defects in adult-onset Krabbe disease.Role of endogenous psychosine accumulation in oligodendrocyte differentiation and survival: implication for Krabbe disease.Weighing the evidence for newborn screening for early-infantile Krabbe disease.Treating inflammation in childhood neurodegenerative disorders.Lysosomal lipid storage diseases.The cellular pathology of lysosomal diseases.Advances and pitfalls of cell therapy in metabolic leukodystrophies.Neuronopathic lysosomal storage diseases: clinical and pathologic findings.Psychosine, a marker of Krabbe phenotype and treatment effect.Lithium improves cell viability in psychosine-treated MO3.13 human oligodendrocyte cell line via autophagy activation.Heterozygote galactocerebrosidase (GALC) mutants have reduced remyelination and impaired myelin debris clearance following demyelinating injury.
P2860
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P2860
description
2003 nî lūn-bûn
@nan
2003 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年学术文章
@wuu
2003年学术文章
@zh-cn
2003年学术文章
@zh-hans
2003年学术文章
@zh-my
2003年学术文章
@zh-sg
2003年學術文章
@yue
name
Globoid cell leukodystrophy (Krabbe's disease): update.
@ast
Globoid cell leukodystrophy (Krabbe's disease): update.
@en
type
label
Globoid cell leukodystrophy (Krabbe's disease): update.
@ast
Globoid cell leukodystrophy (Krabbe's disease): update.
@en
prefLabel
Globoid cell leukodystrophy (Krabbe's disease): update.
@ast
Globoid cell leukodystrophy (Krabbe's disease): update.
@en
P2860
P1476
Globoid cell leukodystrophy (Krabbe's disease): update.
@en
P2093
Kunihiko Suzuki
P2860
P304
P356
10.1177/08830738030180090201
P577
2003-09-01T00:00:00Z