HUDA ZOGHBI to Cerebellum
This is a "connection" page, showing publications HUDA ZOGHBI has written about Cerebellum.
Connection Strength
5.898
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Atoh1 drives the heterogeneity of the pontine nuclei neurons and promotes their differentiation. Sci Adv. 2023 06 30; 9(26):eadg1671.
Score: 0.599
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Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1. J Clin Invest. 2022 05 02; 132(9).
Score: 0.553
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Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1. EMBO J. 2021 04 01; 40(7):e106106.
Score: 0.511
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Deleting Mecp2 from the cerebellum rather than its neuronal subtypes causes a delay in motor learning in mice. Elife. 2021 01 26; 10.
Score: 0.507
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Neurexophilin4 is a selectively expressed a-neurexin ligand that modulates specific cerebellar synapses and motor functions. Elife. 2019 09 16; 8.
Score: 0.461
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Pontocerebellar hypoplasia: review of classification and genetics, and exclusion of several genes known to be important for cerebellar development. J Child Neurol. 2011 Mar; 26(3):288-94.
Score: 0.255
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In vivo Atoh1 targetome reveals how a proneural transcription factor regulates cerebellar development. Proc Natl Acad Sci U S A. 2011 Feb 22; 108(8):3288-93.
Score: 0.254
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Deletion of Atoh1 disrupts Sonic Hedgehog signaling in the developing cerebellum and prevents medulloblastoma. Science. 2009 Dec 04; 326(5958):1424-7.
Score: 0.234
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Mouse models of MeCP2 disorders share gene expression changes in the cerebellum and hypothalamus. Hum Mol Genet. 2009 Jul 01; 18(13):2431-42.
Score: 0.224
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ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell. 2006 Dec 29; 127(7):1335-47.
Score: 0.191
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Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum. Neuron. 2005 Oct 06; 48(1):31-43.
Score: 0.175
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Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1. Neuron. 2023 02 15; 111(4):481-492.e8.
Score: 0.145
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Genetic regulation of cerebellar development. Nat Rev Neurosci. 2001 Jul; 2(7):484-91.
Score: 0.130
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PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1. Hum Mol Genet. 2018 08 15; 27(16):2863-2873.
Score: 0.107
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Loss of Atoh1 from neurons regulating hypoxic and hypercapnic chemoresponses causes neonatal respiratory failure in mice. Elife. 2018 07 04; 7.
Score: 0.106
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ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism. Neuron. 2018 03 21; 97(6):1235-1243.e5.
Score: 0.104
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Math1 is essential for genesis of cerebellar granule neurons. Nature. 1997 Nov 13; 390(6656):169-72.
Score: 0.101
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The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Nature. 1997 Oct 30; 389(6654):974-8.
Score: 0.101
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Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways. Neuron. 2016 Mar 16; 89(6):1194-1207.
Score: 0.090
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A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1. Elife. 2015 May 19; 4.
Score: 0.085
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Pharmacometabolomic signature of ataxia SCA1 mouse model and lithium effects. PLoS One. 2013; 8(8):e70610.
Score: 0.075
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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: 0.074
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Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua. Science. 2011 Nov 04; 334(6056):690-3.
Score: 0.067
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Gcn5 loss-of-function accelerates cerebellar and retinal degeneration in a SCA7 mouse model. Hum Mol Genet. 2012 Jan 15; 21(2):394-405.
Score: 0.067
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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: 0.061
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Phosphorylation of ATXN1 at Ser776 in the cerebellum. J Neurochem. 2009 Jul; 110(2):675-86.
Score: 0.056
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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: 0.054
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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: 0.048
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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: 0.044
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Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1. Neuron. 2024 Feb 07; 112(3):362-383.e15.
Score: 0.039
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Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1. Hum Mol Genet. 2003 Nov 01; 12(21):2789-95.
Score: 0.038
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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. Neuron. 2003 Feb 06; 37(3):383-401.
Score: 0.036
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Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles. Neuron. 2023 02 15; 111(4):493-507.e6.
Score: 0.036
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Dissection of the cellular and molecular events that position cerebellar Purkinje cells: a study of the math1 null-mutant mouse. J Neurosci. 2002 Sep 15; 22(18):8110-6.
Score: 0.035
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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: 0.034
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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: 0.033
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Proprioceptor pathway development is dependent on Math1. Neuron. 2001 May; 30(2):411-22.
Score: 0.032
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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: 0.028
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Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. J Neurosci. 1997 Oct 01; 17(19):7385-95.
Score: 0.025
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Disruption of the ATXN1-CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans. Nat Genet. 2017 Apr; 49(4):527-536.
Score: 0.024
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Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1. J Neurosci. 2013 May 29; 33(22):9328-36.
Score: 0.019
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Isolation, mapping, and characterization of two cDNA clones expressed in the cerebellum. Genomics. 1992 Nov; 14(3):813-5.
Score: 0.018
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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: 0.015
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SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 1995 Sep 22; 82(6):937-48.
Score: 0.005