Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathies - Wikidata - awgldk - TriplyDB

Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathies

Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathies

http://www.wikidata.org/entity/Q36653957

about

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 Glucocerebrosidase and Parkinson disease: Recent advances 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 The relationship between glucocerebrosidase mutations and Parkinson disease Mitochondrial dysfunction associated with glucocerebrosidase deficiency LIMP-2 expression is critical for β-glucocerebrosidase activity and α-synuclein clearance Glucocerebrosidase modulates cognitive and motor activities in murine models of Parkinson's disease.The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.Comparison of Parkinson risk in Ashkenazi Jewish patients with Gaucher disease and GBA heterozygotes.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.Structural features of membrane-bound glucocerebrosidase and α-synuclein probed by neutron reflectometry and fluorescence spectroscopy.A GCase chaperone improves motor function in a mouse model of synucleinopathy.Gaucher-Associated Parkinsonism.Glucocerebrosidase Deficiency in Drosophila Results in α-Synuclein-Independent Protein Aggregation and Neurodegeneration.Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice.Combination therapies: The next logical Step for the treatment of synucleinopathies?Glucocerebrosidase in the pathogenesis and treatment of Parkinson disease.GBA deficiency promotes SNCA/α-synuclein accumulation through autophagic inhibition by inactivated PPP2A.Atp13a2-deficient mice exhibit neuronal ceroid lipofuscinosis, limited α-synuclein accumulation and age-dependent sensorimotor deficits.Characterization of the complex formed by β-glucocerebrosidase and the lysosomal integral membrane protein type-2.The Autophagy-Lysosomal Pathway in Neurodegeneration: A TFEB Perspective Activation of β-Glucocerebrosidase Reduces Pathological α-Synuclein and Restores Lysosomal Function in Parkinson's Patient Midbrain Neurons.Parkinson disease-linked GBA mutation effects reversed by molecular chaperones in human cell and fly models.Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models.Progress and potential of non-inhibitory small molecule chaperones for the treatment of Gaucher disease and its implications for Parkinson disease.The association between ß-glucocerebrosidase mutations and parkinsonism Glucocerebrosidase mutations and the pathogenesis of Parkinson disease.Gene therapy for the neurological manifestations in lysosomal storage disorders.Genetics and genomics of Parkinson's disease.Genetic convergence of Parkinson's disease and lysosomal storage disorders.Autophagy in neuronal cells: general principles and physiological and pathological functions.The ongoing pursuit of neuroprotective therapies in Parkinson disease.Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.Therapeutic potential of autophagy-enhancing agents in Parkinson's disease.Glucocerebrosidase in Parkinson's disease: Insights into pathogenesis and prospects for treatment.

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Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathies

http://www.wikidata.org/entity/Q36653957

description

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2013年论文

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2013年论文

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name

Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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type

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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

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Augmenting CNS glucocerebrosid ...... cher-related synucleinopathies

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Catherine Viel

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Christopher M Treleaven

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James C Dodge

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Jennifer Clarke

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Jie Bu

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Lamya S Shihabuddin

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Lindsay Sweet

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Marco A Passini

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Monyrath Chan

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Richard L Sidman

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P2860

Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathies

Multicenter analysis of glucocerebrosidase mutations in Parkinson's disease.

Glucocerebrosidase mutations in clinical and pathologically proven Parkinson's disease

Molecular pathways of neurodegeneration in Parkinson's disease

CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy.

GBA mutations increase risk for Lewy body disease with and without Alzheimer disease pathology.

Concurrence of alpha-synuclein and tau brain pathology in the Contursi kindred.

The link between the GBA gene and parkinsonism

Colocalization of tau and alpha-synuclein epitopes in Lewy bodies.

Direct observation of the interconversion of normal and toxic forms of α-synuclein

P304

3537-3542

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10.1073/PNAS.1220464110

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9

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110

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2013-01-07T00:00:00Z

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234086995

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23297226

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Parkinson's disease

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3587272

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