Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.
about
Diagnostic workup and management of patients with suspected Niemann-Pick type C diseasePathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathwayFostering collaborative research for rare genetic disease: the example of niemann-pick type C diseaseIntracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinDevelopment of targeted therapies for Parkinson's disease and related synucleinopathiesCholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1)Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.Immune dysfunction in Niemann-Pick disease type C.Electrodiagnostic testing and histopathologic changes confirm peripheral nervous system myelin abnormalities in the feline model of niemann-pick disease type C.Normalisation of brain spectroscopy findings in Niemann-Pick disease type C patients treated with miglustat.β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profilingMolecular characterization and in vitro differentiation of feline progenitor-like amniotic epithelial cells.The potential of histone deacetylase inhibitors in Niemann - Pick type C disease.Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1.Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1.Relative acidic compartment volume as a lysosomal storage disorder-associated biomarkerInhibition of GM3 synthase attenuates neuropathology of Niemann-Pick disease Type C. by affecting sphingolipid metabolism.Sphingolipid lysosomal storage disorders.Complex lipid trafficking in Niemann-Pick disease type C.Autophagic activity in neuronal cell deathN-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.Crosstalk between sphingolipids and vitamin D3: potential role in the nervous system.Impact of miglustat on evolution of atypical presentation of late-infantile-onset Niemann-Pick disease type C with early cognitive impairment, behavioral dysfunction, epilepsy, ophthalmoplegia, and cerebellar involvement: a case report.Longitudinal changes in cerebellar and subcortical volumes in adult-onset Niemann-Pick disease type C patients treated with miglustat.Precision Medicine in Cats: Novel Niemann-Pick Type C1 Diagnosed by Whole-Genome Sequencing2-hydroxypropyl-β-cyclodextrins and the Blood-Brain Barrier: Considerations for Niemann-Pick Disease Type C1.Longitudinal Changes in White Matter Fractional Anisotropy in Adult-Onset Niemann-Pick Disease Type C Patients Treated with Miglustat.Delivering drugs to the central nervous system: an overview.Inhibition of glucosylceramide synthase stimulates autophagy flux in neurons.Miglustat in Niemann-Pick disease type C patients: a review
P2860
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P2860
Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@ast
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@en
type
label
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@ast
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@en
prefLabel
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@ast
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@en
P2093
P2860
P1476
Miglustat improves purkinje ce ...... e Niemann-Pick disease type C.
@en
P2093
Alexandra Crooks
Caroline Bryan
Charles H Vite
Jasper Dingemanse
Maria Prociuk
Marie T Vanier
Patricia O'Donnell
Steven U Walkley
Tracey Sikora
Veronika M Stein
P2860
P304
P356
10.1097/NEN.0B013E31825414A6
P577
2012-05-01T00:00:00Z