Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2 - Wikidata - awgldk - TriplyDB

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

http://www.wikidata.org/entity/Q36477656

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Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease Integration of modeling with experimental and clinical findings synthesizes and refines the central role of inositol 1,4,5-trisphosphate receptor 1 in spinocerebellar ataxia Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse model Motor Dysfunctions and Neuropathology in Mouse Models of Spinocerebellar Ataxia Type 2: A Comprehensive Review Essential tremor: a neurodegenerative disease?Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay(ji-hes) mice Spinocerebellar ataxia type 19/22 mutations alter heterocomplex Kv4.3 channel function and gating in a dominant manner A positive feedback loop linking enhanced mGluR function and basal calcium in spinocerebellar ataxia type 2 Cerebellar zonal patterning relies on Purkinje cell neurotransmission.Understanding essential tremor: progress on the biological front From neurons to neuron neighborhoods: the rewiring of the cerebellar cortex in essential tremor.Computational neurobiology is a useful tool in translational neurology: the example of ataxia The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders.Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.Chronic suppression of inositol 1,4,5-triphosphate receptor-mediated calcium signaling in cerebellar purkinje cells alleviates pathological phenotype in spinocerebellar ataxia 2 mice Spontaneous shaker rat mutant - a new model for X-linked tremor/ataxia.Degenerative ataxias, from genes to therapies: The 2015 Cotzias Lecture.In vivo analysis of cerebellar Purkinje cell activity in SCA2 transgenic mouse model.4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6.Purkinje cell axonal anatomy: quantifying morphometric changes in essential tremor versus control brains.New roles for the cerebellum in health and disease.Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.From mice to men: lessons from mutant ataxic mice.Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders.Cellular and circuit mechanisms underlying spinocerebellar ataxias.Cerebellar ataxias: β-III spectrin's interactions suggest common pathogenic pathways.Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia.Prodromal spinocerebellar ataxia type 2: Prospects for early interventions and ethical challenges.Atxn2 Knockout and CAG42-Knock-in Cerebellum Shows Similarly Dysregulated Expression in Calcium Homeostasis Pathway.Downregulation of Purkinje Cell Activity by Modulators of Small Conductance Calcium-Activated Potassium Channels In Rat Cerebellum.Antisense oligonucleotide therapy for spinocerebellar ataxia type 2.Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2.Polyglutamine spinocerebellar ataxias - from genes to potential treatments.Climbing Fiber Development Is Impaired in Postnatal Car8 (wdl) Mice.ATXN2-AS, a gene antisense to ATXN2, is associated with spinocerebellar ataxia type 2 and amyotrophic lateral sclerosis.Transient cerebellar alterations during development prior to obvious motor phenotype in a mouse model of spinocerebellar ataxia type 6.Ion channel dysfunction in cerebellar ataxia.Potassium channel dysfunction underlies Purkinje neuron spiking abnormalities in spinocerebellar ataxia type 2.Targeting potassium channels to treat cerebellar ataxia.Transcriptional Dysregulation and Post-translational Modifications in Polyglutamine Diseases: From Pathogenesis to Potential Therapeutic Strategies.

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Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

http://www.wikidata.org/entity/Q36477656

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Human Molecular Genetics

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Changes in Purkinje cell firin ...... ology in a mouse model of SCA2

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Pratap Meera

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Stefan M Pulst

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Stephen T Hansen

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Thomas S Otis

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P2860

A novel protein with RNA-binding motifs interacts with ataxin-2

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes

Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules

Ataxin-2 associates with the endocytosis complex and affects EGF receptor trafficking

Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1

Episodic ataxia/myokymia syndrome is associated with point mutations in the human potassium channel gene, KCNA1

KCNC3: phenotype, mutations, channel biophysics-a study of 260 familial ataxia patients

Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals

A Drosophila homolog of the polyglutamine disease gene SCA2 is a dosage-sensitive regulator of actin filament formation

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271-283

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10.1093/HMG/DDS427

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2

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22

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23087021

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3526159

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