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ZOGHBI, HUDA

One or more keywords matched the following items that are connected to ZOGHBI, HUDA

Item Type	Name
Academic Article	A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.
Academic Article	The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up.
Academic Article	Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice.
Academic Article	Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.
Academic Article	Identification of a novel phosphorylation site in ataxin-1.
Academic Article	Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.
Academic Article	RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice.
Academic Article	SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.
Academic Article	dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1.
Academic Article	miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.
Academic Article	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.
Academic Article	Phosphorylation of ATXN1 at Ser776 in the cerebellum.
Academic Article	Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration.
Academic Article	SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal.
Academic Article	ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization.
Academic Article	The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins.
Academic Article	ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.
Academic Article	Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.
Academic Article	RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.
Academic Article	The role of LANP and ataxin 1 in E4F-mediated transcriptional repression.
Academic Article	CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.
Academic Article	Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.
Academic Article	The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7.
Academic Article	Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1.
Academic Article	Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.
Academic Article	Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1.
Academic Article	Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis.
Academic Article	Inactivation of hnRNP K by expanded intronic AUUCU repeat induces apoptosis via translocation of PKCdelta to mitochondria in spinocerebellar ataxia 10.
Academic Article	Gcn5 loss-of-function accelerates cerebellar and retinal degeneration in a SCA7 mouse model.
Academic Article	Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua.
Academic Article	Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxia.
Academic Article	14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus.
Academic Article	Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.
Academic Article	Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.
Academic Article	Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice.
Academic Article	Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice.
Academic Article	A cell-based screen for modulators of ataxin-1 phosphorylation.
Academic Article	Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model.
Academic Article	Characterization of the zebrafish atxn1/axh gene family.
Academic Article	SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.
Academic Article	Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model.
Academic Article	Ataxin1L is a regulator of HSC function highlighting the utility of cross-tissue comparisons for gene discovery.
Academic Article	Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1.
Academic Article	Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels.
Academic Article	A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1.
Academic Article	Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes.
Academic Article	Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.
Academic Article	Disruption of the ATXN1-CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans.
Academic Article	Motor neuron degeneration correlates with respiratory dysfunction in SCA1.
Academic Article	ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism.
Academic Article	Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model.
Academic Article	PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1.
Academic Article	Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles.
Concept	Ataxin-1
Concept	Ataxin-7
Concept	Ataxin-10
Academic Article	Loss of Ataxin-1 Potentiates Alzheimer's Pathogenesis by Elevating Cerebral BACE1 Transcription.
Academic Article	miR760 regulates ATXN1 levels via interaction with its 5' untranslated region.
Academic Article	Modulation of ATXN1 S776 phosphorylation reveals the importance of allele-specific targeting in SCA1.
Academic Article	Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1.
Academic Article	A Structural Study of the Cytoplasmic Chaperone Effect of 14-3-3 Proteins on Ataxin-1.
Academic Article	Reduction of mutant ATXN1 rescues premature death in a conditional SCA1 mouse model.
Academic Article	Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1.
Academic Article	Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles.
Academic Article	Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1.
Academic Article	Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1.

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Ataxin 7