Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
about
Identification of a molecular target of psychosine and its role in globoid cell formation.New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperonesLysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular ToxicityCombined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy.Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophyPsychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.Multi-system disorders of glycosphingolipid and ganglioside metabolismFactors that affect postnatal bone growth retardation in the twitcher murine model of Krabbe disease.Globoid cell leukodystrophy (Krabbe's disease): update.Effect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease.Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosidesPersistence 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 leukodystrophyPeroxisomal dysfunction in inflammatory childhood white matter disorders: an unexpected contributor to neuropathologyMutations in the fatty acid 2-hydroxylase gene are associated with leukodystrophy with spastic paraparesis and dystonia.Early 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.Effect of intrastriatal mesenchymal stromal cell injection on progression of a murine model of Krabbe disease.Secondary lipid accumulation in lysosomal disease.Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3.Arylsulfatase A deficiency causes seminolipid accumulation and a lysosomal storage disorder in Sertoli cellsBone 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.Mesenchymal lineage stem cells have pronounced anti-inflammatory effects in the twitcher mouse model of Krabbe's diseaseAstrocytes and lysosomal storage diseases.Elevated globotriaosylsphingosine is a hallmark of Fabry disease.Lysosomal Storage Diseases-Regulating Neurodegeneration.The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors.Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.Psychosine-triggered endomitosis is modulated by membrane sphingolipids through regulation of phosphoinositide 4,5-bisphosphate production at the cleavage furrow.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.Psychosine-induced alterations in peroxisomes of twitcher mouse liverConcise Synthesis of the Unnatural Sphingosine and Psychosine Enantiomer.Pervasive supply of therapeutic lysosomal enzymes in the CNS of normal and Krabbe-affected non-human primates by intracerebral lentiviral gene therapyCharacterization and application of a disease-cell model for a neurodegenerative lysosomal disease.Lipid and Glycolipid Isomer Analyses Using Ultra-High Resolution Ion Mobility Spectrometry SeparationsNeuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.Advances and pitfalls of cell therapy in metabolic leukodystrophies.My journey into the world of sphingolipids and sphingolipidoses.My encounters with Krabbe disease: A personal recollection of a 40-Year journey with young colleagues.
P2860
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P2860
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
description
1984 nî lūn-bûn
@nan
1984年の論文
@ja
1984年論文
@yue
1984年論文
@zh-hant
1984年論文
@zh-hk
1984年論文
@zh-mo
1984年論文
@zh-tw
1984年论文
@wuu
1984年论文
@zh
1984年论文
@zh-cn
name
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
@en
type
label
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
@en
prefLabel
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
@en
P356
P1433
P1476
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
@en
P2093
P304
P356
10.1126/SCIENCE.6719111
P407
P577
1984-05-01T00:00:00Z