Published in Development on May 01, 2002
Functional auditory hair cells produced in the mammalian cochlea by in utero gene transfer. Nature (2008) 3.21
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Wnt5a functions in planar cell polarity regulation in mice. Dev Biol (2007) 2.85
Smaller inner ear sensory epithelia in Neurog 1 null mice are related to earlier hair cell cycle exit. Dev Dyn (2005) 2.71
The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear. PLoS Genet (2006) 2.56
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Neurod1 regulates survival and formation of connections in mouse ear and brain. Cell Tissue Res (2010) 1.12
Differentiation of the lateral compartment of the cochlea requires a temporally restricted FGF20 signal. PLoS Biol (2012) 1.10
Canonical Notch signaling is not necessary for prosensory induction in the mouse cochlea: insights from a conditional mutant of RBPjkappa. J Neurosci (2011) 1.09
Canal cristae growth and fiber extension to the outer hair cells of the mouse ear require Prox1 activity. PLoS One (2010) 1.08
The Atoh1-lineage gives rise to hair cells and supporting cells within the mammalian cochlea. Dev Biol (2013) 1.07
Hey2 functions in parallel with Hes1 and Hes5 for mammalian auditory sensory organ development. BMC Dev Biol (2008) 1.07
Regeneration of stereocilia of hair cells by forced Atoh1 expression in the adult mammalian cochlea. PLoS One (2012) 1.06
Transcription factors define the neuroanatomical organization of the medullary reticular formation. Front Neuroanat (2013) 1.03
Single-cell RNA-Seq resolves cellular complexity in sensory organs from the neonatal inner ear. Nat Commun (2015) 1.03
Differential and overlapping expression pattern of SOX2 and SOX9 in inner ear development. Gene Expr Patterns (2009) 1.02
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Diverse expression patterns of LIM-homeodomain transcription factors (LIM-HDs) in mammalian inner ear development. Dev Dyn (2008) 1.01
Manipulating cell cycle regulation in the mature cochlea. Hear Res (2007) 1.01
Barhl1 regulatory sequences required for cell-specific gene expression and autoregulation in the inner ear and central nervous system. Mol Cell Biol (2008) 1.00
Regulation of cochlear convergent extension by the vertebrate planar cell polarity pathway is dependent on p120-catenin. Development (2012) 1.00
Atoh1, an essential transcription factor in neurogenesis and intestinal and inner ear development: function, regulation, and context dependency. J Assoc Res Otolaryngol (2012) 0.99
Continued expression of GATA3 is necessary for cochlear neurosensory development. PLoS One (2013) 0.99
Conditional deletion of Atoh1 reveals distinct critical periods for survival and function of hair cells in the organ of Corti. J Neurosci (2013) 0.99
Conditional deletion of N-Myc disrupts neurosensory and non-sensory development of the ear. Dev Dyn (2011) 0.98
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Notch signaling limits supporting cell plasticity in the hair cell-damaged early postnatal murine cochlea. PLoS One (2013) 0.97
Sox2 and Fgf interact with Atoh1 to promote sensory competence throughout the zebrafish inner ear. Dev Biol (2011) 0.97
Notch signaling and Atoh1 expression during hair cell regeneration in the mouse utricle. Hear Res (2010) 0.96
Characterization of the transcriptome of nascent hair cells and identification of direct targets of the Atoh1 transcription factor. J Neurosci (2015) 0.95
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Stemness of the organ of Corti relates to the epigenetic status of Sox2 enhancers. PLoS One (2012) 0.95
Overlapping and distinct pRb pathways in the mammalian auditory and vestibular organs. Cell Cycle (2011) 0.95
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Jxc1/Sobp, encoding a nuclear zinc finger protein, is critical for cochlear growth, cell fate, and patterning of the organ of corti. J Neurosci (2008) 0.94
Overactivation of Notch1 signaling induces ectopic hair cells in the mouse inner ear in an age-dependent manner. PLoS One (2012) 0.93
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Mouse models for dissecting vertebrate planar cell polarity signaling in the inner ear. Brain Res (2009) 0.92
Functional features of trans-differentiated hair cells mediated by Atoh1 reveals a primordial mechanism. J Neurosci (2012) 0.92
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Sonic hedgehog (SHH) promotes the differentiation of mouse cochlear neural progenitors via the Math1-Brn3.1 signaling pathway in vitro. J Neurosci Res (2010) 0.91
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Evolution and development of the tetrapod auditory system: an organ of Corti-centric perspective. Evol Dev (2013) 0.91
Basic helix-loop-helix gene Hes6 delineates the sensory hair cell lineage in the inner ear. Dev Dyn (2006) 0.91
Identification and subclassification of new Atoh1 derived cell populations during mouse spinal cord development. Dev Biol (2008) 0.90
TFE2 and GATA3 enhance induction of POU4F3 and myosin VIIa positive cells in nonsensory cochlear epithelium by ATOH1. Dev Biol (2012) 0.90
C-MYC transcriptionally amplifies SOX2 target genes to regulate self-renewal in multipotent otic progenitor cells. Stem Cell Reports (2014) 0.89
Inner ear development: building a spiral ganglion and an organ of Corti out of unspecified ectoderm. Cell Tissue Res (2014) 0.89
Identification of genes concordantly expressed with Atoh1 during inner ear development. Anat Cell Biol (2011) 0.89
Inner ear hair cell-like cells from human embryonic stem cells. Stem Cells Dev (2014) 0.89
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Conditional gene expression in the mouse inner ear using Cre-loxP. J Assoc Res Otolaryngol (2012) 0.89
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Atoh1 expression and function during auditory hair cell regeneration in post-hatch chickens. Hear Res (2012) 0.87
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MeCP2, a key contributor to neurological disease, activates and represses transcription. Science (2008) 10.11
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A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. Cell (2006) 8.61
The story of Rett syndrome: from clinic to neurobiology. Neuron (2007) 8.08
Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes. Nature (2010) 5.11
Diseases of unstable repeat expansion: mechanisms and common principles. Nat Rev Genet (2005) 4.66
Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet (2004) 4.45
MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron (2007) 4.15
Isolation of neural stem cells from the postnatal cerebellum. Nat Neurosci (2005) 3.97
Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome. J Neurosci (2006) 3.69
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature (2008) 3.68
Genetic fate mapping reveals that the caudal ganglionic eminence produces a large and diverse population of superficial cortical interneurons. J Neurosci (2010) 3.61
Math1-driven GFP expression in the developing nervous system of transgenic mice. Gene Expr Patterns (2003) 3.42
ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell (2006) 3.39
Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation. Hum Mol Genet (2002) 3.12
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Gfi1 functions downstream of Math1 to control intestinal secretory cell subtype allocation and differentiation. Genes Dev (2005) 2.94
Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum. Neuron (2005) 2.93
Regulation of RNA splicing by the methylation-dependent transcriptional repressor methyl-CpG binding protein 2. Proc Natl Acad Sci U S A (2005) 2.90
RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell (2006) 2.80
Intestine-specific ablation of mouse atonal homolog 1 (Math1) reveals a role in cellular homeostasis. Gastroenterology (2007) 2.79
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Increased MECP2 gene copy number as the result of genomic duplication in neurodevelopmentally delayed males. Genet Med (2006) 2.78
MeCP2 dysfunction in Rett syndrome and related disorders. Curr Opin Genet Dev (2006) 2.71
Mammalian cochlear supporting cells can divide and trans-differentiate into hair cells. Nature (2006) 2.65
Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndrome. Hum Mol Genet (2004) 2.61
Mash1 specifies neurons and oligodendrocytes in the postnatal brain. EMBO J (2004) 2.55
Complex rearrangements in patients with duplications of MECP2 can occur by fork stalling and template switching. Hum Mol Genet (2009) 2.50
Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell (2003) 2.50
Progressive hearing loss in mice lacking the cyclin-dependent kinase inhibitor Ink4d. Nat Cell Biol (2003) 2.46
Autoregulation of neurogenesis by GDF11. Neuron (2003) 2.40
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron (2003) 2.39
The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival. Development (2003) 2.38
Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress. Neuron (2008) 2.36
Specification of dorsal spinal cord interneurons. Curr Opin Neurobiol (2003) 2.33
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Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A (2006) 2.25
Merkel cells are essential for light-touch responses. Science (2009) 2.24
Math1 is essential for the development of hindbrain neurons critical for perinatal breathing. Neuron (2009) 2.23
A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome. Hum Mol Genet (2008) 2.13
Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction. PLoS Biol (2006) 2.12
Hey2 regulation by FGF provides a Notch-independent mechanism for maintaining pillar cell fate in the organ of Corti. Dev Cell (2009) 2.11
Adult neural function requires MeCP2. Science (2011) 2.09
The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell (2005) 2.09
Autism and other neuropsychiatric symptoms are prevalent in individuals with MeCP2 duplication syndrome. Ann Neurol (2009) 2.07
Protein interactome reveals converging molecular pathways among autism disorders. Sci Transl Med (2011) 2.04
The E-protein Tcf4 interacts with Math1 to regulate differentiation of a specific subset of neuronal progenitors. Proc Natl Acad Sci U S A (2007) 2.01
Mouse models of MeCP2 disorders share gene expression changes in the cerebellum and hypothalamus. Hum Mol Genet (2009) 2.00
A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration. Neuron (2002) 1.97
Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua. Science (2011) 1.96
Inverted genomic segments and complex triplication rearrangements are mediated by inverted repeats in the human genome. Nat Genet (2011) 1.93
Ptf1a determines GABAergic over glutamatergic neuronal cell fate in the spinal cord dorsal horn. Development (2005) 1.90
miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis. Nat Neurosci (2008) 1.89
SHANK3 overexpression causes manic-like behaviour with unique pharmacogenetic properties. Nature (2013) 1.86
Loss of MeCP2 in aminergic neurons causes cell-autonomous defects in neurotransmitter synthesis and specific behavioral abnormalities. Proc Natl Acad Sci U S A (2009) 1.79
Cross-regulation of Ngn1 and Math1 coordinates the production of neurons and sensory hair cells during inner ear development. Development (2007) 1.76
Inactivation of hnRNP K by expanded intronic AUUCU repeat induces apoptosis via translocation of PKCdelta to mitochondria in spinocerebellar ataxia 10. PLoS Genet (2010) 1.76
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. Hum Mol Genet (2005) 1.73
A minimal Ksp-cadherin promoter linked to a green fluorescent protein reporter gene exhibits tissue-specific expression in the developing kidney and genitourinary tract. J Am Soc Nephrol (2002) 1.72
A morphogenetic wave of p27Kip1 transcription directs cell cycle exit during organ of Corti development. Development (2006) 1.71
Human-specific regulation of MeCP2 levels in fetal brains by microRNA miR-483-5p. Genes Dev (2013) 1.70
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X-chromosome inactivation patterns are unbalanced and affect the phenotypic outcome in a mouse model of rett syndrome. Am J Hum Genet (2004) 1.69
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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) 1.64
Ascl1 defines sequentially generated lineage-restricted neuronal and oligodendrocyte precursor cells in the spinal cord. Development (2006) 1.63
Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice. Hum Mol Genet (2004) 1.61
Overexpression of methyl-CpG binding protein 2 impairs T(H)1 responses. Sci Transl Med (2012) 1.56
SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776. Neuron (2010) 1.56
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J Biol Chem (2006) 1.55
A nonclassical bHLH Rbpj transcription factor complex is required for specification of GABAergic neurons independent of Notch signaling. Genes Dev (2008) 1.54
In vivo analysis of Ascl1 defined progenitors reveals distinct developmental dynamics during adult neurogenesis and gliogenesis. J Neurosci (2007) 1.54
Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model. PLoS Med (2007) 1.52
Solving the autism puzzle a few pieces at a time. Neuron (2011) 1.48
Expression of Islet1 marks the sensory and neuronal lineages in the mammalian inner ear. J Comp Neurol (2004) 1.45
In vivo Atoh1 targetome reveals how a proneural transcription factor regulates cerebellar development. Proc Natl Acad Sci U S A (2011) 1.44
Aberrant myofibril assembly in tropomodulin1 null mice leads to aborted heart development and embryonic lethality. J Cell Biol (2003) 1.44
Distinct domains within Mash1 and Math1 are required for function in neuronal differentiation versus neuronal cell-type specification. Development (2004) 1.43
Deletion of Atoh1 disrupts Sonic Hedgehog signaling in the developing cerebellum and prevents medulloblastoma. Science (2009) 1.42
Ascl1 (Mash1) defines cells with long-term neurogenic potential in subgranular and subventricular zones in adult mouse brain. PLoS One (2011) 1.42
Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis. PLoS Genet (2010) 1.42
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Notch signaling augments the canonical Wnt pathway to specify the size of the otic placode. Development (2008) 1.42
Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1. J Neurosci (2013) 1.41
Rett syndrome: a prototypical neurodevelopmental disorder. Neuroscientist (2004) 1.40
dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet (2007) 1.38
Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels. Proc Natl Acad Sci U S A (2008) 1.34
Sequential roles for Mash1 and Ngn2 in the generation of dorsal spinal cord interneurons. Development (2005) 1.34
Excitatory neurons of the proprioceptive, interoceptive, and arousal hindbrain networks share a developmental requirement for Math1. Proc Natl Acad Sci U S A (2009) 1.32
Ascl1 (Mash1) lineage cells contribute to discrete cell populations in CNS architecture. Mol Cell Neurosci (2008) 1.31
Transcriptional autoregulation controls pancreatic Ptf1a expression during development and adulthood. Mol Cell Biol (2008) 1.31
Neuroepithelial body microenvironment is a niche for a distinct subset of Clara-like precursors in the developing airways. Proc Natl Acad Sci U S A (2012) 1.31
Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes. Nat Genet (2007) 1.30
Mapmodulin/leucine-rich acidic nuclear protein binds the light chain of microtubule-associated protein 1B and modulates neuritogenesis. J Biol Chem (2003) 1.28