Axonopathy is a compounding factor in the pathogenesis of Krabbe disease. - Wikidata - awgldk - TriplyDB

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

http://www.wikidata.org/entity/Q43118014

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Myelin damage and repair in pathologic CNS: challenges and prospects Lysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular Toxicity Combined 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 leukodystrophy Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture.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.Regional differences in fiber tractography predict neurodevelopmental outcomes in neonates with infantile Krabbe disease.Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3.Neural stem cell gene therapy ameliorates pathology and function in a mouse model of globoid cell leukodystrophy.Design of a regulated lentiviral vector for hematopoietic stem cell gene therapy of globoid cell leukodystrophy Astrocytes and lysosomal storage diseases.The 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 disease Inhibition 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.Multipotent stromal cells alleviate inflammation, neuropathology, and symptoms associated with globoid cell leukodystrophy in the twitcher mouse.A microglial hypothesis of globoid cell leukodystrophy pathology.How membrane dysfunction influences neuronal survival pathways in sphingolipid storage disorders Fluid levity of the cell: Role of membrane lipid architecture in genetic sphingolipidoses.Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.Patient fibroblasts-derived induced neurons demonstrate autonomous neuronal defects in adult-onset Krabbe disease.Advances and pitfalls of cell therapy in metabolic leukodystrophies.Synaptic failure: The achilles tendon of sphingolipidoses Inherited and acquired disorders of myelin: The underlying myelin pathology.Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease.Biochemical, cell biological, pathological, and therapeutic aspects of Krabbe's disease.Axonal pathology in Krabbe's disease: The cytoskeleton as an emerging therapeutic target.Treatment for Krabbe's disease: Finding the combination.Perspective on innovative therapies for globoid cell leukodystrophy.Psychosine induces the dephosphorylation of neurofilaments by deregulation of PP1 and PP2A phosphatases.Detection of the neurotoxin psychosine in samples of peripheral blood: application in diagnostics and follow-up of Krabbe disease.Beyond Krabbe's disease: The potential contribution of galactosylceramidase deficiency to neuronal vulnerability in late-onset synucleinopathies Region- and age-dependent alterations of glial-neuronal metabolic interactions correlate with CNS pathology in a mouse model of globoid cell leukodystrophy.Primary bone marrow mesenchymal stromal cells rescue the axonal phenotype of Twitcher mice.Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.Long-Term Improvement of Neurological Signs and Metabolic Dysfunction in a Mouse Model of Krabbe's Disease after Global Gene Therapy.Analysis of age-related changes in psychosine metabolism in the human brain.α-Synuclein interacts directly but reversibly with psychosine: implications for α-synucleinopathies

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P2860

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

http://www.wikidata.org/entity/Q43118014

description

2011 nî lūn-bûn

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2011年の論文

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年學術文章

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2011年學術文章

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name

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@en

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@nl

type

label

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@en

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@nl

prefLabel

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@en

Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

@nl

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Acta Neuropathologica

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Axonopathy is a compounding factor in the pathogenesis of Krabbe disease

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Aurora Lopez-Rosas

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Benjamin Smith

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Ernesto Roque Bongarzone

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Hongling Zhu

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Ludovico Cantuti Castelvetri

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Maria Irene Givogri

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Xi Qiu

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P2860

Identification of programmed cell death in situ via specific labeling of nuclear DNA fragmentation

Dynamics of Kv1 channel transport in axons

Krabbe disease: genetic aspects and progress toward therapy

Transplantation of umbilical-cord blood in babies with infantile Krabbe's disease

The organization of the endoplasmic reticulum and the intermediate compartment in cultured rat hippocampal neurons

Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of a neurodegenerative disease

Imaging neuronal subsets in transgenic mice expressing multiple spectral variants of GFP

Genes involved in leukodystrophies: a glance at glial functions.

Early and severe sensory loss in three adult siblings with hexosaminidase A and B deficiency (Sandhoff disease).

The synthesis and transport of lipids for axonal growth and nerve regeneration.

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1

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