CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy - Wikidata - awgldk - TriplyDB

CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy

CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy

http://www.wikidata.org/entity/Q35122963

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Genetic perspective on the role of the autophagy-lysosome pathway in Parkinson disease Reduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson's disease Glucocerebrosidase is shaking up the synucleinopathies Imiglucerase in the treatment of Gaucher disease: a history and perspective Glucocerebrosidase and Parkinson disease: Recent advances Protein degradation pathways in Parkinson's disease: curse or blessing Viable neuronopathic Gaucher disease model in Medaka (Oryzias latipes) displays axonal accumulation of alpha-synuclein iPSC-derived dopamine neurons reveal differences between monozygotic twins discordant for Parkinson's disease Mitochondrial dysfunction associated with glucocerebrosidase deficiency Loss of β-glucocerebrosidase activity does not affect alpha-synuclein levels or lysosomal function in neuronal cells The p.L302P mutation in the lysosomal enzyme gene SMPD1 is a risk factor for Parkinson disease Visualization of Active Glucocerebrosidase in Rodent Brain with High Spatial Resolution following In Situ Labeling with Fluorescent Activity Based Probes Modelling idiopathic Parkinson disease as a complex illness can inform incidence rate in healthy adults: the PREDIGT score Glucocerebrosidase modulates cognitive and motor activities in murine models of Parkinson's disease.Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice Comparison of Parkinson risk in Ashkenazi Jewish patients with Gaucher disease and GBA heterozygotes.Visual short-term memory deficits associated with GBA mutation and Parkinson's disease.Gaucher disease: insights from a rare Mendelian disorder.The link between the GBA gene and parkinsonism Emerging insights into the mechanistic link between α-synuclein and glucocerebrosidase in Parkinson's disease Development of targeted therapies for Parkinson's disease and related synucleinopathies The LIMP-2/SCARB2 binding motif on acid β-glucosidase: basic and applied implications for Gaucher disease and associated neurodegenerative diseases.Augmentation of phenotype in a transgenic Parkinson mouse heterozygous for a Gaucher mutation Structural features of membrane-bound glucocerebrosidase and α-synuclein probed by neutron reflectometry and fluorescence spectroscopy.Loss of glucocerebrosidase 1 activity causes lysosomal dysfunction and α-synuclein aggregation.Dissociation of glucocerebrosidase dimer in solution by its co-factor, saposin C.A GCase chaperone improves motor function in a mouse model of synucleinopathy.Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.Glucocerebrosidase depletion enhances cell-to-cell transmission of α-synuclein.Did α-Synuclein and Glucocerebrosidase Coevolve? Implications for Parkinson's Disease.Gaucher-Associated Parkinsonism.Lysosomal Enzyme Glucocerebrosidase Protects against Aβ1-42 Oligomer-Induced Neurotoxicity.Cognitive performance of GBA mutation carriers with early-onset PD: the CORE-PD study.Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice.Glucocerebrosidase activity in Parkinson's disease with and without GBA mutations Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Glucocerebrosidase mutations alter the endoplasmic reticulum and lysosomes in Lewy body 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.

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CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy

http://www.wikidata.org/entity/Q35122963

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2011 nî lūn-bûn

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2011 թուականի Յուլիսին հրատարակուած գիտական յօդուած

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2011年の論文

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2011年論文

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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Proceedings of the National Academy of Sciences of the United States of America

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CNS expression of glucocerebro ...... aucher-related synucleinopathy

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Cathrine Kinnecom

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Gregory A Grabowski

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Jennifer Clarke

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Lamya S Shihabuddin

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Lingyun Li

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Lisa M Stanek

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Marco A Passini

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Richard L Sidman

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S Pablo Sardi

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Seng H Cheng

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P2860

Misfolded proteinase K-resistant hyperphosphorylated alpha-synuclein in aged transgenic mice with locomotor deterioration and in human alpha-synucleinopathies

Multicenter analysis of glucocerebrosidase mutations in Parkinson's disease.

Glucocerebrosidase mutations in clinical and pathologically proven Parkinson's disease

Alpha-synuclein in Lewy bodies

Neuroinflammation in Parkinson's disease: a target for neuroprotection?

Murine models of acute neuronopathic Gaucher disease.

Glucocerebrosidase gene mutations: a risk factor for Lewy body disorders.

Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease.

Synucleinopathies: clinical and pathological implications.

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Michael G Schlossmacher

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