HUDA ZOGHBI to Ataxins
This is a "connection" page, showing publications HUDA ZOGHBI has written about Ataxins.
Connection Strength
2.171
-
RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature. 2013 Jun 20; 498(7454):325-331.
Score: 0.098
-
Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua. Science. 2011 Nov 04; 334(6056):690-3.
Score: 0.088
-
ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization. Dev Cell. 2011 Oct 18; 21(4):746-57.
Score: 0.088
-
Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proc Natl Acad Sci U S A. 2011 Feb 01; 108(5):2142-7.
Score: 0.083
-
Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis. PLoS Genet. 2010 Jul 08; 6(7):e1001021.
Score: 0.080
-
Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1. J Biol Chem. 2009 Mar 20; 284(12):7425-9.
Score: 0.071
-
miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis. Nat Neurosci. 2008 Oct; 11(10):1137-9.
Score: 0.071
-
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature. 2008 Apr 10; 452(7188):713-8.
Score: 0.068
-
The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7. Proc Natl Acad Sci U S A. 2008 Jan 29; 105(4):1291-6.
Score: 0.068
-
Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model. PLoS Med. 2007 May; 4(5):e182.
Score: 0.064
-
Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes. Nat Genet. 2007 Mar; 39(3):373-9.
Score: 0.063
-
ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell. 2006 Dec 29; 127(7):1335-47.
Score: 0.063
-
The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell. 2005 Aug 26; 122(4):633-44.
Score: 0.057
-
Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell. 2003 May 16; 113(4):457-68.
Score: 0.049
-
A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration. Neuron. 2002 Jun 13; 34(6):905-19.
Score: 0.046
-
Reduction of Purkinje cell pathology in SCA1 transgenic mice by p53 deletion. Neurobiol Dis. 2001 Dec; 8(6):974-81.
Score: 0.044
-
SCA1 molecular genetics: a history of a 13 year collaboration against glutamines. Hum Mol Genet. 2001 Oct 01; 10(20):2307-11.
Score: 0.044
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum Mol Genet. 2001 Jul 01; 10(14):1511-8.
Score: 0.043
-
Spinocerebellar ataxias. Neurobiol Dis. 2000 Oct; 7(5):523-7.
Score: 0.041
-
Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus. Hum Mol Genet. 2000 Mar 22; 9(5):779-85.
Score: 0.039
-
Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1. Nat Neurosci. 2000 Feb; 3(2):157-63.
Score: 0.039
-
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron. 1999 Dec; 24(4):879-92.
Score: 0.038
-
Progress in pathogenesis studies of spinocerebellar ataxia type 1. Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29; 354(1386):1079-81.
Score: 0.037
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat Genet. 1998 Jun; 19(2):148-54.
Score: 0.035
-
The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Nature. 1997 Oct 30; 389(6654):974-8.
Score: 0.033
-
The expansion of the CAG repeat in ataxin-2 is a frequent cause of autosomal dominant spinocerebellar ataxia. Neurology. 1997 Oct; 49(4):1009-13.
Score: 0.033
-
The expanding world of ataxins. Nat Genet. 1996 Nov; 14(3):237-8.
Score: 0.031
-
Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase. Hum Mol Genet. 1996 Sep; 5(9):1311-8.
Score: 0.031
-
Analysis of the CAG repeat and gene product in spinocerebellar ataxia type 1. Proc Assoc Am Physicians. 1995 Jul; 107(2):231-6.
Score: 0.028
-
Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nat Genet. 1995 May; 10(1):94-8.
Score: 0.028
-
Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels. Cell. 2015 Mar 12; 160(6):1087-98.
Score: 0.028
-
Identification and characterization of the gene causing type 1 spinocerebellar ataxia. Nat Genet. 1994 Aug; 7(4):513-20.
Score: 0.027
-
Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1. J Neurosci. 2013 May 29; 33(22):9328-36.
Score: 0.024
-
Ataxin1L is a regulator of HSC function highlighting the utility of cross-tissue comparisons for gene discovery. PLoS Genet. 2013 Mar; 9(3):e1003359.
Score: 0.024
-
Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum. J Neurosci. 2013 Mar 27; 33(13):5806-20.
Score: 0.024
-
Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua. Genes Dev. 2013 Mar 15; 27(6):590-5.
Score: 0.024
-
14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus. J Biol Chem. 2011 Oct 07; 286(40):34606-16.
Score: 0.022
-
Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model. Hum Mol Genet. 2011 Jun 01; 20(11):2204-12.
Score: 0.021
-
SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776. Neuron. 2010 Sep 23; 67(6):929-35.
Score: 0.020
-
Phosphorylation of ATXN1 at Ser776 in the cerebellum. J Neurochem. 2009 Jul; 110(2):675-86.
Score: 0.019
-
Characterization of the zebrafish atxn1/axh gene family. J Neurogenet. 2009; 23(3):313-23.
Score: 0.018
-
dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet. 2007 Dec 28; 3(12):e234.
Score: 0.017
-
The role of LANP and ataxin 1 in E4F-mediated transcriptional repression. EMBO Rep. 2007 Jul; 8(7):671-7.
Score: 0.016
-
Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1. J Neurochem. 2007 Sep; 102(6):2040-2048.
Score: 0.016
-
RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell. 2006 Nov 17; 127(4):697-708.
Score: 0.016
-
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J Biol Chem. 2006 Sep 08; 281(36):26714-24.
Score: 0.015
-
SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal. J Biol Chem. 2005 Jun 10; 280(23):21942-8.
Score: 0.014
-
A cell-based screen for modulators of ataxin-1 phosphorylation. Hum Mol Genet. 2005 Apr 15; 14(8):1095-105.
Score: 0.014
-
Identification of a novel phosphorylation site in ataxin-1. Biochim Biophys Acta. 2005 May 15; 1744(1):11-8.
Score: 0.014
-
Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice. J Neurosci. 2004 Oct 06; 24(40):8853-61.
Score: 0.013
-
Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice. Hum Mol Genet. 2004 Oct 15; 13(20):2535-43.
Score: 0.013
-
The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up. J Biol Chem. 2004 Oct 01; 279(40):42290-301.
Score: 0.013
-
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron. 2003 May 08; 38(3):375-87.
Score: 0.012
-
Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice. Neuromolecular Med. 2002; 1(1):33-42.
Score: 0.011
-
Altered trafficking of membrane proteins in purkinje cells of SCA1 transgenic mice. Am J Pathol. 2001 Sep; 159(3):905-13.
Score: 0.011
-
Calcium dynamics and electrophysiological properties of cerebellar Purkinje cells in SCA1 transgenic mice. J Neurophysiol. 2001 Apr; 85(4):1750-60.
Score: 0.011
-
The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract. Hum Mol Genet. 2001 Jan 01; 10(1):25-30.
Score: 0.010
-
Identification of genes that modify ataxin-1-induced neurodegeneration. Nature. 2000 Nov 02; 408(6808):101-6.
Score: 0.010
-
Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. Hum Mol Genet. 2000 Sep 22; 9(15):2305-12.
Score: 0.010
-
Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell. 1998 Oct 02; 95(1):41-53.
Score: 0.009
-
Close associations between prevalences of dominantly inherited spinocerebellar ataxias with CAG-repeat expansions and frequencies of large normal CAG alleles in Japanese and Caucasian populations. Am J Hum Genet. 1998 Oct; 63(4):1060-6.
Score: 0.009
-
Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation. J Neurosci. 1998 Jul 15; 18(14):5508-16.
Score: 0.009
-
Interruptions in the triplet repeats of SCA1 and FRAXA reduce the propensity and complexity of slipped strand DNA (S-DNA) formation. Biochemistry. 1998 Feb 24; 37(8):2701-8.
Score: 0.009
-
Increased trinucleotide repeat instability with advanced maternal age. Hum Mol Genet. 1997 Nov; 6(12):2135-9.
Score: 0.008
-
Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures. Nature. 1997 Oct 30; 389(6654):971-4.
Score: 0.008
-
Identification of a self-association region within the SCA1 gene product, ataxin-1. Hum Mol Genet. 1997 Apr; 6(4):513-8.
Score: 0.008
-
Cloning and developmental expression analysis of the murine homolog of the spinocerebellar ataxia type 1 gene (Sca1). Hum Mol Genet. 1996 Jan; 5(1):33-40.
Score: 0.007
-
Mapping of the Sca1 and pcd genes on mouse chromosome 13 provides evidence that they are different genes. Genomics. 1995 Oct 10; 29(3):812-3.
Score: 0.007
-
SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 1995 Sep 22; 82(6):937-48.
Score: 0.007