Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1.
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Bergmann glial S100B activates myo-inositol monophosphatase 1 and Co-localizes to purkinje cell vacuoles in SCA1 transgenic miceCurrent understanding of the role of microRNAs in spinocerebellar ataxiasPolyglutamine expansion accelerates the dynamics of ataxin-1 and does not result in aggregate formation.Self-Assembly and Conformational Heterogeneity of the AXH Domain of Ataxin-1: An Unusual Example of a Chameleon FoldTargeting several CAG expansion diseases by a single antisense oligonucleotideThe design and delivery of a thermally responsive peptide to inhibit S100B-mediated neurodegenerationInhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice.Dopamine D2 receptor signaling modulates mutant ataxin-1 S776 phosphorylation and aggregation.FOX-2 dependent splicing of ataxin-2 transcript is affected by ataxin-1 overexpressionFunctionally enigmatic genes: a case study of the brain ignorome.The vasculome of the mouse brain.Glial S100B protein modulates mutant ataxin-1 aggregation and toxicity: TRTK12 peptide, a potential candidate for SCA1 therapyFocused cerebellar laser light induced hyperthermia improves symptoms and pathology of polyglutamine disease SCA1 in a mouse model.Ataxin-1 poly(Q)-induced proteotoxic stress and apoptosis are attenuated in neural cells by docosahexaenoic acid-derived neuroprotectin D1.The importance of serine 776 in Ataxin-1 partner selection: a FRET analysis.Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.Glial response to polyglutamine-mediated stress.Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.Mapping the self-association domains of ataxin-1: identification of novel non overlapping motifs.Modifiers and mechanisms of multi-system polyglutamine neurodegenerative disorders: lessons from fly models.Polyglutamine (PolyQ) diseases: genetics to treatments.Penetrating the cell membrane, thermal targeting and novel anticancer drugs: the development of thermally targeted, elastin-like polypeptide cancer therapeutics.Mesenchymal stem cells as a potential therapeutic tool for spinocerebellar ataxia.Spinal Cord Damage in Spinocerebellar Ataxia Type 1.Mesenchymal stem cells ameliorate cerebellar pathology in a mouse model of spinocerebellar ataxia type 1.Ataxin-1 regulates the cerebellar bioenergetics proteome through the GSK3β-mTOR pathway which is altered in Spinocerebellar ataxia type 1 (SCA1).Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy.Loss of intrinsic organization of cerebellar networks in spinocerebellar ataxia type 1: correlates with disease severity and duration.The design and delivery of a PKA inhibitory polypeptide to treat SCA1.Spinal cord atrophy in spinocerebellar ataxia type 1.Knockdown of acid-sensing ion channel 1a (ASIC1a) suppresses disease phenotype in SCA1 mouse model.Metabolic evidence for cerebral neurodegeneration in spinocerebellar ataxia type 1.m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration.Mesenchymal stem cells attenuate peripheral neuronal degeneration in spinocerebellar ataxia type 1 knockin mice.A novel function of Ataxin-1 in the modulation of PP2A activity is dysregulated in the spinocerebellar ataxia type 1.Increased catabolic state in spinocerebellar ataxia type 1 patients.Morphological and Functional Attenuation of Degeneration of Peripheral Neurons by Mesenchymal Stem Cell-Conditioned Medium in Spinocerebellar Ataxia Type 1-Knock-in Mice.Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model.Suppression of calbindin-D28k expression exacerbates SCA1 phenotype in a disease mouse model.
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Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on January 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
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vědecký článek
@cs
name
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@en
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@nl
type
label
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@en
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@nl
prefLabel
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@en
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1.
@nl
P2860
P1433
P1476
Clinical, genetic, molecular, ...... spinocerebellar ataxia type 1
@en
P2093
Robert Goold
P2860
P2888
P304
P356
10.1007/S12311-008-0009-0
P577
2008-01-01T00:00:00Z