Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis (Q28593098)

Capicua transcriptional repressor

GOA release 2020-03-11

1 reference

transcriptional dysregulation

GOA release 2020-03-11

1 reference

based on heuristic

inferred from title

spinocerebellar ataxia type 1

1 reference

5

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Chad Shaw

series ordinal

3

object named as

Chad A. Shaw

1 reference

31 January 2017

Juan Crespo-Barreto

1

1 reference

31 January 2017

John D. Fryer

series ordinal

2

1 reference

31 January 2017

Harry T. Orr

series ordinal

4

1 reference

31 January 2017

8 July 2010

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31 January 2017

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31 January 2017

6

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31 January 2017

7

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31 January 2017

e1001021

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31 January 2017

Creative Commons Attribution 4.0 International

start time

8 July 2010

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

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on focus list of Wikimedia project

ScienceSource

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cites work

MeCP2, a key contributor to neurological disease, activates and represses transcription

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Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

17 March 2017

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The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7

17 March 2017

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17 March 2017

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RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice

17 March 2017

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Generation and characterization of Sca2 (ataxin-2) knockout mice

17 March 2017

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The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins

17 March 2017

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Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1.

17 March 2017

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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

17 March 2017

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Climbing fiber activation of EAAT4 transporters and kainate receptors in cerebellar Purkinje cells

17 March 2017

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RORalpha coordinates reciprocal signaling in cerebellar development through sonic hedgehog and calcium-dependent pathways

17 March 2017

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Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

17 March 2017

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Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

17 March 2017

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Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

17 March 2017

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Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation

17 March 2017

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Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

17 March 2017

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Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

17 March 2017

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Trinucleotide Repeat Disorders

17 March 2017

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Characterization of the zebrafish atxn1/axh gene family

6 April 2017

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Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels

29 September 2017

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29 September 2017

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Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease

29 September 2017

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Diseases of unstable repeat expansion: mechanisms and common principles

29 September 2017

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Generation and characterization of androgen receptor knockout (ARKO) mice: an in vivo model for the study of androgen functions in selective tissues

29 September 2017

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Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

29 September 2017

1 reference

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Type 1 inositol 1,4,5-trisphosphate receptor knock-out mice: their phenotypes and their meaning in neuroscience and clinical practice

29 September 2017

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X-linked recessive bulbospinal neuronopathy (SBMA).

29 September 2017

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Deficient cerebellar long-term depression and impaired motor learning in mGluR1 mutant mice

29 September 2017

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Spinocerebellar ataxia type 1.

29 September 2017

1 reference

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Fusion between CIC and DUX4 up-regulates PEA3 family genes in Ewing-like sarcomas with t(4;19)(q35;q13) translocation

29 September 2017

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Mitotic stability and meiotic variability of the (CAG)n repeat in the Huntington disease gene

3 June 2018

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The status of SCA1, MJD/SCA3, FRDA, DRPLA and MD triplet containing genes in patients with Huntington disease and healthy controls

3 June 2018

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SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.

21 January 2018

1 reference

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A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.

21 January 2018

1 reference

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Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice

21 January 2018

1 reference

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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

21 January 2018

1 reference

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ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Rotorod sensorimotor learning in cerebellar mutant mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Exacerbated synucleinopathy in mice expressing A53T SNCA on a Snca null background

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Loss of presenilin function causes Alzheimer's disease-like neurodegeneration in the mouse.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

The expansion of the CAG repeat in ataxin-2 is a frequent cause of autosomal dominant spinocerebellar ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PGEN.1001021

Loss of endogenous androgen receptor protein accelerates motor neuron degeneration and accentuates androgen insensitivity in a mouse model of X-linked spinal and bulbar muscular atrophy

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20628574