Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy
about
Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient miceAstrocytes are central in the pathomechanisms of vanishing white matterDeveloping therapeutic approaches for metachromatic leukodystrophyDeletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease miceGeneralized glycogen storage and cardiomegaly in a knockout mouse model of Pompe diseaseNeurological deficits and glycosphingolipid accumulation in saposin B deficient mice.Gene therapy for metachromatic leukodystrophy.Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.Correction of metachromatic leukodystrophy in the mouse model by transplantation of genetically modified hematopoietic stem cells.Sulfatide with short fatty acid dominates in astrocytes and neurons.Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophyMulti-system disorders of glycosphingolipid and ganglioside metabolismInterferons, signal transduction pathways, and the central nervous system.The mousetrap: what we can learn when the mouse model does not mimic the human disease.Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIBModest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene.Gene therapy of metachromatic leukodystrophy reverses neurological damage and deficits in mice.Loss of central auditory processing in a mouse model of Canavan disease.Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometryUbiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Metachromatic leukodystrophy: recent research developments.The myelin mutants as models to study myelin repair in the leukodystrophies.Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiencyRemodeling of the plasma membrane in preparation for sperm-egg recognition: roles of acrosomal proteins.Enzyme replacement in the CSF to treat metachromatic leukodystrophy in mouse model using single intracerebroventricular injection of self-complementary AAV1 vectorGene therapy of metachromatic leukodystrophy.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Correction of brain oligodendrocytes by AAVrh.10 intracerebral gene therapy in metachromatic leukodystrophy mice.Brain conditioning is instrumental for successful microglia reconstitution following hematopoietic stem cell transplantationSphingolipids and membrane biology as determined from genetic models.Anti-inflammatory Therapy With Simvastatin Improves Neuroinflammation and CNS Function in a Mouse Model of Metachromatic LeukodystrophyArylsulfatase A Overexpressing Human iPSC-derived Neural Cells Reduce CNS Sulfatide Storage in a Mouse Model of Metachromatic Leukodystrophy.Novel candidate disease for gene therapy: metachromatic leukodystrophy.Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.Development of gene therapy for blood disorders: an update.Rapid and Efficient Stable Gene Transfer to Mesenchymal Stromal Cells Using a Modified Foamy Virus Vector.Factors and processes modulating phenotypes in neuronopathic lysosomal storage diseases.Myelin lesions associated with lysosomal and peroxisomal disorders.Lysosomal lipid storage diseases.Advances and pitfalls of cell therapy in metabolic leukodystrophies.
P2860
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P2860
Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy
description
1996 nî lūn-bûn
@nan
1996 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
name
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@ast
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@en
type
label
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@ast
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@en
prefLabel
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@ast
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@en
P2093
P2860
P356
P1476
Phenotype of arylsulfatase A-d ...... n metachromatic leukodystrophy
@en
P2093
D Hartmann
H H Goebel
K von Figura
P2860
P304
14821-14826
P356
10.1073/PNAS.93.25.14821
P407
P577
1996-12-01T00:00:00Z