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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	Mapmodulin/leucine-rich acidic nuclear protein binds the light chain of microtubule-associated protein 1B and modulates neuritogenesis.
Academic Article	Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum.
Academic Article	Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3.
Academic Article	A mutant form of MeCP2 protein associated with human Rett syndrome cannot be displaced from methylated DNA by notch in Xenopus embryos.
Academic Article	Molecular neuroscience: BAC-to-BAC images of the brain.
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	MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number.
Academic Article	Failure of neuronal homeostasis results in common neuropsychiatric phenotypes.
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	Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei.
Academic Article	Deletion of Atoh1 disrupts Sonic Hedgehog signaling in the developing cerebellum and prevents medulloblastoma.
Academic Article	Excitatory neurons of the proprioceptive, interoceptive, and arousal hindbrain networks share a developmental requirement for Math1.
Academic Article	In vivo neuronal subtype-specific targets of Atoh1 (Math1) in dorsal spinal cord.
Academic Article	Atoh1 governs the migration of postmitotic neurons that shape respiratory effectiveness at birth and chemoresponsiveness in adulthood.
Academic Article	CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.
Academic Article	Cell-specific expression of wild-type MeCP2 in mouse models of Rett syndrome yields insight about pathogenesis.
Academic Article	The story of Rett syndrome: from clinic to neurobiology.
Academic Article	Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress.
Academic Article	Math1 is essential for the development of hindbrain neurons critical for perinatal breathing.
Academic Article	Loss of MeCP2 in aminergic neurons causes cell-autonomous defects in neurotransmitter synthesis and specific behavioral abnormalities.
Academic Article	Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes.
Academic Article	In vivo Atoh1 targetome reveals how a proneural transcription factor regulates cerebellar development.
Academic Article	Neurogenetics: advancing the "next-generation" of brain research.
Academic Article	Solving the autism puzzle a few pieces at a time.
Academic Article	Human-specific regulation of MeCP2 levels in fetal brains by microRNA miR-483-5p.
Academic Article	Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation.
Academic Article	Serine 776 of ataxin-1 is critical for polyglutamine-induced 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	Postnatal neurodevelopmental disorders: meeting at the synapse?
Academic Article	The E-protein Tcf4 interacts with Math1 to regulate differentiation of a specific subset of neuronal progenitors.
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	Ataxin1L is a regulator of HSC function highlighting the utility of cross-tissue comparisons for gene discovery.
Concept	Neurons
Concept	Motor Neurons
Academic Article	Dendritic arborization and spine dynamics are abnormal in the mouse model of MECP2 duplication syndrome.
Academic Article	Atoh1-dependent rhombic lip neurons are required for temporal delay between independent respiratory oscillators in embryonic mice.
Academic Article	MeCP2 binds to non-CG methylated DNA as neurons mature, influencing transcription and the timing of onset for Rett syndrome.
Academic Article	Rett syndrome: disruption of epigenetic control of postnatal neurological functions.
Academic Article	MECP2 disorders: from the clinic to mice and back.
Academic Article	Loss of MeCP2 in Parvalbumin-and Somatostatin-Expressing Neurons in Mice Leads to Distinct Rett Syndrome-like Phenotypes.
Academic Article	Manipulations of MeCP2 in glutamatergic neurons highlight their contributions to Rett and other neurological disorders.
Academic Article	Restoration of Mecp2 expression in GABAergic neurons is sufficient to rescue multiple disease features in a mouse model of Rett syndrome.
Academic Article	Loss and Gain of MeCP2 Cause Similar Hippocampal Circuit Dysfunction that Is Rescued by Deep Brain Stimulation in a Rett Syndrome Mouse Model.
Academic Article	Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.
Academic Article	Reduction of Nuak1 Decreases Tau and Reverses Phenotypes in a Tauopathy Mouse Model.
Academic Article	Respiratory Network Stability and Modulatory Response to Substance P Require Nalcn.
Academic Article	A Mild PUM1 Mutation Is Associated with Adult-Onset Ataxia, whereas Haploinsufficiency Causes Developmental Delay and Seizures.
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	A Druggable Genome Screen Identifies Modifiers of a-Synuclein Levels via a Tiered Cross-Species Validation Approach.
Academic Article	Loss of Atoh1 from neurons regulating hypoxic and hypercapnic chemoresponses causes neonatal respiratory failure in mice.
Academic Article	A kinome-wide RNAi screen identifies ERK2 as a druggable regulator of Shank3 stability.
Grant	MOLECULAR STUDIES OF SPINOCEREBELLAR ATAXIS TYPE I
Grant	MOLECULAR PATHOGENESIS STUDIES OF RETT SYNDROME
Concept	Cholinergic Neurons
Concept	GABAergic Neurons
Academic Article	Neurexophilin4 is a selectively expressed a-neurexin ligand that modulates specific cerebellar synapses and motor functions.
Academic Article	The distinct methylation landscape of maturing neurons and its role in Rett syndrome pathogenesis.
Academic Article	Mecp2 Deletion from Cholinergic Neurons Selectively Impairs Recognition Memory and Disrupts Cholinergic Modulation of the Perirhinal Cortex.
Academic Article	Doublecortin-like Kinase 1 Regulates a-Synuclein Levels and Toxicity.
Academic Article	Optimizing Nervous System-Specific Gene Targeting with Cre Driver Lines: Prevalence of Germline Recombination and Influencing Factors.
Academic Article	Losing Dnmt3a dependent methylation in inhibitory neurons impairs neural function by a mechanism impacting Rett syndrome.
Academic Article	Partial loss of CFIm25 causes learning deficits and aberrant neuronal alternative polyadenylation.
Academic Article	Coexpression enrichment analysis at the single-cell level reveals convergent defects in neural progenitor cells and their cell-type transitions in neurodevelopmental disorders.
Academic Article	MeCP2 Levels Regulate the 3D Structure of Heterochromatic Foci in Mouse Neurons.
Academic Article	Deleting Mecp2 from the cerebellum rather than its neuronal subtypes causes a delay in motor learning in mice.
Academic Article	Identification and characterization of conserved noncoding cis-regulatory elements that impact Mecp2 expression and neurological functions.
Academic Article	Presymptomatic training mitigates functional deficits in a mouse model of Rett syndrome.
Academic Article	Maturation of Purkinje cell firing properties relies on neurogenesis of excitatory neurons.
Academic Article	A weakened recurrent circuit in the hippocampus of Rett syndrome mice disrupts long-term memory representations.
Academic Article	Reduction of mutant ATXN1 rescues premature death in a conditional SCA1 mouse model.
Academic Article	Disruption of MeCP2-TCF20 complex underlies distinct neurodevelopmental disorders.
Academic Article	Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles.
Academic Article	Evolutionarily conserved regulators of tau identify targets for new therapies.
Academic Article	The Transcription Factor Pou3f1 Sheds Light on the Development and Molecular Diversity of Glutamatergic Cerebellar Nuclear Neurons in the Mouse.
Academic Article	Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1.
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	A novel pathogenic mutation of MeCP2 impairs chromatin association independent of protein levels.
Academic Article	Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1.

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Neurons