Murine models of acute neuronopathic Gaucher disease. - Wikidata - wikimedia - TriplyDB

Murine models of acute neuronopathic Gaucher disease.

Murine models of acute neuronopathic Gaucher disease.

http://www.wikidata.org/entity/Q30480646

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Glucocerebrosidase is shaking up the synucleinopathies Glucocerebrosidase 1 deficient Danio rerio mirror key pathological aspects of human Gaucher disease and provide evidence of early microglial activation preceding alpha-synuclein-independent neuronal cell death Viable neuronopathic Gaucher disease model in Medaka (Oryzias latipes) displays axonal accumulation of alpha-synuclein The relationship between glucocerebrosidase mutations and Parkinson disease Mitochondrial dysfunction associated with glucocerebrosidase deficiency In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models Systemic delivery of a glucosylceramide synthase inhibitor reduces CNS substrates and increases lifespan in a mouse model of type 2 Gaucher disease Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's disease A Novel Functional Missense Mutation p.T219A in Type 1 Gaucher's Disease.The saccadic and neurological deficits in type 3 Gaucher disease.Multi-system disorders of glycosphingolipid and ganglioside metabolism Neuronopathic Gaucher's disease: induced pluripotent stem cells for disease modelling and testing chaperone activity of small compounds.Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease.Identification of Wolbachia-responsive microRNAs in the two-spotted spider mite, Tetranychus urticae.Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice.Defective Self-Renewal and Differentiation of GBA-Deficient Neural Stem Cells Can Be Restored By Macrophage Colony-Stimulating Factor.Neuroinflammatory paradigms in lysosomal storage diseases.Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model Astrocytes and lysosomal storage diseases.Neonatal Bone Marrow Transplantation in MPS IIIA Mice.Induction of the type I interferon response in neurological forms of Gaucher disease β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.Covalent inhibitors of glycosidases and their applications in biochemistry and biology.Lysosomal impairment in Parkinson's disease.Autophagic lysosome reformation dysfunction in glucocerebrosidase deficient cells: relevance to Parkinson disease Neuropathic Gaucher disease.Animal models for Gaucher disease research.Mutant GBA1 expression and synucleinopathy risk: first insights from cellular and mouse models.Emerging therapeutic targets for Gaucher disease.Glucosylsphingosine promotes α-synuclein pathology in mutant GBA-associated Parkinson's disease.Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.Gaucher disease: Progress and ongoing challenges.Induced Pluripotent Stem Cell Modeling of Gaucher's Disease: What Have We Learned?Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits Glucocerebrosidase Mutations in Parkinson Disease.Necroptosis in neurodegenerative diseases: a potential therapeutic target.

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Murine models of acute neuronopathic Gaucher disease.

http://www.wikidata.org/entity/Q30480646

description

2007 nî lūn-bûn

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2007 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

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2007 թվականի հոտեմբերին հրատարակված գիտական հոդված

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2007年の論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

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2007年论文

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name

Murine models of acute neuronopathic Gaucher disease.

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Murine models of acute neuronopathic Gaucher disease.

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Murine models of acute neuronopathic Gaucher disease.

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Murine models of acute neuronopathic Gaucher disease.

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Murine models of acute neuronopathic Gaucher disease.

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Murine models of acute neuronopathic Gaucher disease.

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PNAS.0708086104

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

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Murine models of acute neuronopathic Gaucher disease.

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P2093

Andreas Ooka

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Christophe Lo Bianco

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Deniz Kirik

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Eva Nilsson

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Ida Berglin Enquist

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Jan-Eric Månsson

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Johan Richter

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Mats Ehinger

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Roscoe O Brady

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Stefan Karlsson

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P2860

Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease

Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure

Essential role for survivin in early brain development

Disruption of neural signal transducer and activator of transcription 3 causes obesity, diabetes, infertility, and thermal dysregulation

Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety

Onset and progression in inherited ALS determined by motor neurons and microglia

Effective cell and gene therapy in a murine model of Gaucher disease.

Type 2 Gaucher disease: 15 new cases and review of the literature.

Microglia as mediators of inflammatory and degenerative diseases.

alpha -Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease.

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10.1073/PNAS.0708086104

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104

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2007-10-22T00:00:00Z

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5890694

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17954912

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Glucosidase, beta, acid

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2077282

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