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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	Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum.
Academic Article	Dissection of the cellular and molecular events that position cerebellar Purkinje cells: a study of the math1 null-mutant mouse.
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	miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.
Academic Article	Phosphorylation of ATXN1 at Ser776 in the cerebellum.
Academic Article	Deletion of Atoh1 disrupts Sonic Hedgehog signaling in the developing cerebellum and prevents medulloblastoma.
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	Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.
Academic Article	Mouse models of MeCP2 disorders share gene expression changes in the cerebellum and hypothalamus.
Academic Article	Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis.
Academic Article	In vivo Atoh1 targetome reveals how a proneural transcription factor regulates cerebellar development.
Academic Article	Pontocerebellar hypoplasia: review of classification and genetics, and exclusion of several genes known to be important for cerebellar development.
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	Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.
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	Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1.
Academic Article	SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.
Concept	Cerebellum
Academic Article	Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1.
Academic Article	Pharmacometabolomic signature of ataxia SCA1 mouse model and lithium effects.
Academic Article	A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1.
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	ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism.
Academic Article	PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1.
Academic Article	Loss of Atoh1 from neurons regulating hypoxic and hypercapnic chemoresponses causes neonatal respiratory failure in mice.
Grant	MOLECULAR STUDIES OF SPINOCEREBELLAR ATAXIS TYPE I
Academic Article	Neurexophilin4 is a selectively expressed a-neurexin ligand that modulates specific cerebellar synapses and motor functions.
Academic Article	Deleting Mecp2 from the cerebellum rather than its neuronal subtypes causes a delay in motor learning in mice.
Academic Article	Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1.
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	Atoh1 drives the heterogeneity of the pontine nuclei neurons and promotes their differentiation.
Academic Article	Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1.

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Cerebellum