Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport. - Wikidata - awgldk - TriplyDB

Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.

Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.

http://www.wikidata.org/entity/Q48435478

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Astrocytes differentially respond to inflammatory autoimmune insults and imbalances of neural activity.Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cell-autonomous toxicity Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease Bergmann glial S100B activates myo-inositol monophosphatase 1 and Co-localizes to purkinje cell vacuoles in SCA1 transgenic mice Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease Astrocytes As the Main Players in Primary Degenerative Disorders of the Human Central Nervous System Charcot-Marie-Tooth disease and intracellular traffic Mouse models of polyglutamine diseases: review and data table. Part I Genetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type C Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7 Reelin is a target of polyglutamine expanded ataxin-7 in human spinocerebellar ataxia type 7 (SCA7) astrocytes.Gcn5 loss-of-function accelerates cerebellar and retinal degeneration in a SCA7 mouse model.Histone acetylation, acetyltransferases, and ataxia--alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disorders Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms.Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration Glial cell lineage expression of mutant ataxin-1 and huntingtin induces developmental and late-onset neuronal pathologies in Drosophila models Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1 Profiling the genes affected by pathogenic TDP-43 in astrocytes Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice Loss of beta-III spectrin leads to Purkinje cell dysfunction recapitulating the behavior and neuropathology of spinocerebellar ataxia type 5 in humans.Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy Neighbor effects of neurons bearing protective transgenes In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects.Dysregulation of system xc(-) expression induced by mutant huntingtin in a striatal neuronal cell line and in R6/2 mice Proteomics of dense core secretory vesicles reveal distinct protein categories for secretion of neuroeffectors for cell-cell communication.Brain region specific pre-synaptic and post-synaptic degeneration are early components of neuropathology in prion disease.Autophagy, inflammation and neurodegenerative disease.What can pluripotent stem cells teach us about neurodegenerative diseases?Cellular fusion for gene delivery to SCA1 affected Purkinje neurons.Glial S100B protein modulates mutant ataxin-1 aggregation and toxicity: TRTK12 peptide, a potential candidate for SCA1 therapy Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.The role of the immune system in triplet repeat expansion diseases Sox9 is critical for suppression of neurogenesis but not initiation of gliogenesis in the cerebellum Widespread and efficient transduction of spinal cord and brain following neonatal AAV injection and potential disease modifying effect in ALS mice.

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Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.

http://www.wikidata.org/entity/Q48435478

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Bergmann glia expression of po ...... impairing glutamate transport.

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Bergmann glia expression of po ...... impairing glutamate transport.

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Albert R La Spada

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Bryce L Sopher

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Christian Schultz

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10.1038/NN1750

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