Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models
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Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathiesReduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson's diseaseGenetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type CViable neuronopathic Gaucher disease model in Medaka (Oryzias latipes) displays axonal accumulation of alpha-synucleinGaucher disease: transcriptome analyses using microarray or mRNA sequencing in a Gba1 mutant mouse model treated with velaglucerase alfa or imigluceraseVisualization of Active Glucocerebrosidase in Rodent Brain with High Spatial Resolution following In Situ Labeling with Fluorescent Activity Based ProbesLIMP-2 expression is critical for β-glucocerebrosidase activity and α-synuclein clearanceGlucocerebrosidase modulates cognitive and motor activities in murine models of Parkinson's disease.Multiple pathogenic proteins implicated in neuronopathic Gaucher disease miceInhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis.CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathyProperties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Membrane-bound α-synuclein interacts with glucocerebrosidase and inhibits enzyme activity.α-Synuclein-induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models.Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathiesGlucocerebrosidase deficiency in substantia nigra of parkinson disease brains.Activation of β-Glucocerebrosidase Reduces Pathological α-Synuclein and Restores Lysosomal Function in Parkinson's Patient Midbrain Neurons.A multicenter study of glucocerebrosidase mutations in dementia with Lewy bodies.Lysosomal impairment in Parkinson's disease.Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models.Animal models for Gaucher disease research.Pathological looping in the synucleinopathies: investigating the link between Parkinson's disease and Gaucher disease.Mutant GBA1 expression and synucleinopathy risk: first insights from cellular and mouse models.α-Synuclein in Parkinson's disease.Glucocerebrosidase involvement in Parkinson disease and other synucleinopathies.Gaucher disease paradigm: from ERAD to comorbidity.Neuronopathic lysosomal storage diseases: clinical and pathologic findings.Glucocerebrosidase mutations and the pathogenesis of Parkinson disease.Genetic convergence of Parkinson's disease and lysosomal storage disorders.Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.New Directions in Gaucher Disease.Unfolded protein response in Gaucher disease: from human to Drosophila.The Role of Lipids Interacting with α-Synuclein in the Pathogenesis of Parkinson's Disease.Delineating pathological pathways in a chemically induced mouse model of Gaucher disease.A lysosomal lair for a pathogenic protein pairThe L444P Gba1 mutation enhances alpha-synuclein induced loss of nigral dopaminergic neurons in mice.CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings.Molecular mechanisms of α-synuclein and GBA1 in Parkinson's disease.iPSC-derived neurons from GBA1-associated Parkinson's disease patients show autophagic defects and impaired calcium homeostasis.
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Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
2010年论文
@zh
2010年论文
@zh-cn
name
Accumulation and distribution ...... f Gaucher disease mouse models
@en
type
label
Accumulation and distribution ...... f Gaucher disease mouse models
@en
prefLabel
Accumulation and distribution ...... f Gaucher disease mouse models
@en
P2093
P2860
P1476
Accumulation and distribution ...... f Gaucher disease mouse models
@en
P2093
P2860
P304
P356
10.1016/J.YMGME.2010.12.014
P577
2010-12-31T00:00:00Z