polyglutamine

ataxin 7 ; Mus musculus







37 Article(s)
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1 33888607 SCA7 Mouse Cerebellar Pathology Reveals Preferential Downregulation of Key Purkinje Cell-Identity Genes and Shared Disease Signature with SCA1 and SCA2. 2021 Jun 2 4
2 34852229 Altered H3 histone acetylation impairs high-fidelity DNA repair to promote cerebellar degeneration in spinocerebellar ataxia type 7. 2021 Nov 30 1
3 30559154 SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models. 2019 Jan 11 2
4 30625334 Cell Death Mechanisms in a Mouse Model of Retinal Degeneration in Spinocerebellar Ataxia 7. 2019 Feb 21 9
5 30381411 Antisense oligonucleotides targeting mutant Ataxin-7 restore visual function in a mouse model of spinocerebellar ataxia type 7. 2018 Oct 31 3
6 25755283 Poly(Q) Expansions in ATXN7 Affect Solubility but Not Activity of the SAGA Deubiquitinating Module. 2015 May 7
7 25859008 Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction. 2015 Jul 15 7
8 24859968 The autophagy/lysosome pathway is impaired in SCA7 patients and SCA7 knock-in mice. 2014 Nov 3
9 24930601 Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7. 2014 Sep 1
10 23197655 Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7. 2013 Mar 1 3
11 23347954 H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse. 2013 Jul 3
12 24160175 Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model. 2013 Oct 27 10
13 21827908 Spinocerebellar ataxia type 7. 2012 1
14 22002997 Gcn5 loss-of-function accelerates cerebellar and retinal degeneration in a SCA7 mouse model. 2012 Jan 15 4
15 21689595 CTCF regulates ataxin-7 expression through promotion of a convergently transcribed, antisense noncoding RNA. 2011 Jun 23 3
16 21970987 The linker histone H1C contributes to the SCA7 nuclear phenotype. 2011 Sep-Oct 3
17 22072678 Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration. 2011 Nov 9 5
18 19909779 Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation. 2010 Jan 5
19 20104018 Autophagy: polyQ toxic fragment turnover. 2010 Feb 5
20 20546728 Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons. 2010 Aug 8
21 20600911 Polyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in spinocerebellar ataxia 7 mouse retina. 2010 Oct 4
22 17646170 Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation. 2007 Oct 12 10
23 18054568 Visceral neuropathy and intestinal pseudo-obstruction in a murine model of a nuclear inclusion disease. 2007 Dec 1
24 16434483 Polyglutamine expansion causes neurodegeneration by altering the neuronal differentiation program. 2006 Mar 1 4
25 16494529 Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction. 2006 Mar 7
26 16936724 Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport. 2006 Oct 1
27 15932940 Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration. 2005 Jun 14 3
28 16297465 Identification and characterization of Spinocerebellar Ataxia Type 7 (SCA7) isoform SCA7b in mice. 2005 Dec 20 1
29 14985428 Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model. 2004 Feb 25 5
30 15494410 Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice. 2004 Dec 31 2
31 14526177 Insights into the molecular basis of polyglutamine neurodegeneration from studies of a spinocerebellar ataxia type 7 mouse model. 2003 6
32 12039035 Cloning and expression analysis of the murine homolog of the spinocerebellar ataxia type 7 (SCA7) gene. 2002 Feb 20 3
33 12077187 Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice. 2002 Jun 15 4
34 12471061 Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice. 2002 Dec 15 4
35 11487572 SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types. 2001 Aug 1 2
36 11580893 Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7. 2001 Sep 27 3
37 11030754 Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice. 2000 Oct 12 3