Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes (Q48081281)

8 February 2018

title

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

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8 February 2018

author

Aaron B. Bowman

1

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8 February 2018

Rodney Samaco

6

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Harry T. Orr

9

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Huda Zoghbi

10

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8 February 2018

author name string

Yung C Lam

2

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8 February 2018

Paymaan Jafar-Nejad

3

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Hung-Kai Chen

4

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Ronald Richman

5

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John D Fryer

7

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Juliette J Kahle

8

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language of work or name

English

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publication date

18 February 2007

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8 February 2018

published in

Nature Genetics

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8 February 2018

volume

39

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issue

3

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page(s)

373-379

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8 February 2018

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

cites work

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Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors

21 January 2018

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

21 January 2018

1 reference

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

21 January 2018

1 reference

A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration

21 January 2018

1 reference

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

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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

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Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

21 January 2018

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Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

21 January 2018

1 reference

A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo

21 January 2018

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Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases

21 January 2018

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Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

21 January 2018

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

21 January 2018

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Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.

21 January 2018

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In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo

21 January 2018

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

21 January 2018

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Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease

21 January 2018

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

21 January 2018

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

21 January 2018

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Beta-synuclein displays an antiapoptotic p53-dependent phenotype and protects neurons from 6-hydroxydopamine-induced caspase 3 activation: cross-talk with alpha-synuclein and implication for Parkinson's disease

21 January 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

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Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

21 January 2018

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Complex formation by the Drosophila MSL proteins: role of the MSL2 RING finger in protein complex assembly

21 January 2018

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

21 January 2018

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inferred from DOI database lookup

7 January 2021

based on heuristic

Normal huntingtin function: an alternative approach to Huntington's disease

1 reference

inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1038/NG1977

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8 February 2018

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8 February 2018