Published in Nat Rev Genet on October 01, 2005
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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
Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila. PLoS Biol (2008) 1.33
Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis. Neurology (2011) 1.32
SRS2 and SGS1 prevent chromosomal breaks and stabilize triplet repeats by restraining recombination. Nat Struct Mol Biol (2009) 1.32
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Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol (2008) 1.22
Natural selection drives the accumulation of amino acid tandem repeats in human proteins. Genome Res (2010) 1.21
A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity. PLoS Genet (2012) 1.20
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Emerging pathogenic pathways in the spinocerebellar ataxias. Curr Opin Genet Dev (2009) 1.17
An origin of DNA replication in the promoter region of the human fragile X mental retardation (FMR1) gene. Mol Cell Biol (2006) 1.16
Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxia. Hum Mol Genet (2010) 1.16
Long tract of untranslated CAG repeats is deleterious in transgenic mice. PLoS One (2011) 1.16
Polyglutamine neurodegeneration: expanded glutamines enhance native functions. Curr Opin Genet Dev (2012) 1.13
Xpa deficiency reduces CAG trinucleotide repeat instability in neuronal tissues in a mouse model of SCA1. Hum Mol Genet (2011) 1.12
Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients. Am J Pathol (2009) 1.12
Convergent transcription through a long CAG tract destabilizes repeats and induces apoptosis. Mol Cell Biol (2010) 1.12
Epigenetic boundaries of tumour suppressor gene promoters: the CTCF connection and its role in carcinogenesis. J Cell Mol Med (2006) 1.11
Milestones in ataxia. Mov Disord (2011) 1.11
Sequencing and analysis of chromosome 1 of Eimeria tenella reveals a unique segmental organization. Genome Res (2007) 1.10
From dynamic combinatorial 'hit' to lead: in vitro and in vivo activity of compounds targeting the pathogenic RNAs that cause myotonic dystrophy. Nucleic Acids Res (2012) 1.10
Cause or Effect: Misregulation of microRNA Pathways in Neurodegeneration. Front Neurosci (2012) 1.09
Diverse effects of individual mismatch repair components on transcription-induced CAG repeat instability in human cells. DNA Repair (Amst) (2009) 1.08
Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disorders. Curr Opin Genet Dev (2014) 1.06
SIFT Indel: predictions for the functional effects of amino acid insertions/deletions in proteins. PLoS One (2013) 1.06
Gene Transfer of Brain-derived Neurotrophic Factor (BDNF) Prevents Neurodegeneration Triggered by FXN Deficiency. Mol Ther (2016) 1.06
Complex minisatellite rearrangements generated in the total or partial absence of Rad27/hFEN1 activity occur in a single generation and are Rad51 and Rad52 dependent. Mol Cell Biol (2006) 1.03
Neuronal SUMOylation: mechanisms, physiology, and roles in neuronal dysfunction. Physiol Rev (2014) 1.02
Beyond junk-variable tandem repeats as facilitators of rapid evolution of regulatory and coding sequences. Genes (Basel) (2012) 1.02
Autophagy and polyglutamine diseases. Prog Neurobiol (2011) 1.02
The role of CELF proteins in neurological disorders. RNA Biol (2010) 1.00
Single-stranded DNA-binding protein in vitro eliminates the orientation-dependent impediment to polymerase passage on CAG/CTG repeats. J Biol Chem (2008) 1.00
Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways. Hum Mol Genet (2011) 1.00
Background-dependent effects of polyglutamine variation in the Arabidopsis thaliana gene ELF3. Proc Natl Acad Sci U S A (2012) 0.99
Rational selection of small molecules that increase transcription through the GAA repeats found in Friedreich's ataxia. FEBS Lett (2006) 0.99
The overdue promise of short tandem repeat variation for heritability. Trends Genet (2014) 0.98
Nucleotide excision repair, mismatch repair, and R-loops modulate convergent transcription-induced cell death and repeat instability. PLoS One (2012) 0.97
Protein SUMOylation in neuropathological conditions. Drug News Perspect (2009) 0.97
DNA mismatch repair complex MutSβ promotes GAA·TTC repeat expansion in human cells. J Biol Chem (2012) 0.97
Transcription-induced DNA toxicity at trinucleotide repeats: double bubble is trouble. Cell Cycle (2011) 0.97
Early and late events induced by polyQ-expanded proteins: identification of a common pathogenic property of polYQ-expanded proteins. J Biol Chem (2010) 0.97
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Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proc Natl Acad Sci U S A (2011) 0.96
Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database. BMC Neurol (2012) 0.96
Global microsatellite content distinguishes humans, primates, animals, and plants. Mol Biol Evol (2009) 0.96
Targeting toxic RNAs that cause myotonic dystrophy type 1 (DM1) with a bisamidinium inhibitor. J Am Chem Soc (2014) 0.96
Thermodynamic description of polymorphism in Q- and N-rich peptide aggregates revealed by atomistic simulation. Biophys J (2009) 0.95
AGG interruptions in (CGG)(n) DNA repeat tracts modulate the structure and thermodynamics of non-B conformations in vitro. Biochemistry (2010) 0.95
Ubiquilin overexpression reduces GFP-polyalanine-induced protein aggregates and toxicity. Exp Cell Res (2007) 0.95
Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110. Cell Death Dis (2013) 0.94
Insights into structure, stability, and toxicity of monomeric and aggregated polyglutamine models from molecular dynamics simulations. Biophys J (2008) 0.94
Topoisomerase 1 and single-strand break repair modulate transcription-induced CAG repeat contraction in human cells. Mol Cell Biol (2011) 0.93
Selective inhibition of MBNL1-CCUG interaction by small molecules toward potential therapeutic agents for myotonic dystrophy type 2 (DM2). Nucleic Acids Res (2011) 0.93
Cell-free cloning of highly expanded CTG repeats by amplification of dimerized expanded repeats. Nucleic Acids Res (2008) 0.92
HRaP: database of occurrence of HomoRepeats and patterns in proteomes. Nucleic Acids Res (2013) 0.92
A comparative proteomic analysis of the simple amino acid repeat distributions in Plasmodia reveals lineage specific amino acid selection. PLoS One (2009) 0.92
Mutation patterns of amino acid tandem repeats in the human proteome. Genome Biol (2006) 0.91
Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract. Nat Med (2012) 0.91
Absence of disturbed axonal transport in spinal and bulbar muscular atrophy. Hum Mol Genet (2011) 0.91
Comparative analyses of human single- and multilocus tandem repeats. Genetics (2008) 0.91
Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy. Brain (2013) 0.91
Genes and pathways affected by CAG-repeat RNA-based toxicity in Drosophila. Hum Mol Genet (2011) 0.91
Amyotrophic lateral sclerosis: update and new developments. Degener Neurol Neuromuscul Dis (2012) 0.91
Towards a proteome-scale map of the human protein-protein interaction network. Nature (2005) 24.76
MeCP2, a key contributor to neurological disease, activates and represses transcription. Science (2008) 10.11
Trinucleotide repeat disorders. Annu Rev Neurosci (2007) 9.45
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
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
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
The role of Math1 in inner ear development: Uncoupling the establishment of the sensory primordium from hair cell fate determination. Development (2002) 3.41
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
The basics of translation. Science (2013) 3.00
Failure of neuronal homeostasis results in common neuropsychiatric phenotypes. Nature (2008) 2.98
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
Medicine. The future of psychiatric research: genomes and neural circuits. Science (2010) 2.79
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
Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndrome. Hum Mol Genet (2004) 2.61
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
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
Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice. J Neurosci (2004) 2.29
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
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
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
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
Oligogenic heterozygosity in individuals with high-functioning autism spectrum disorders. Hum Mol Genet (2011) 1.70
Human-specific regulation of MeCP2 levels in fetal brains by microRNA miR-483-5p. Genes Dev (2013) 1.70
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
Preclinical research in Rett syndrome: setting the foundation for translational success. Dis Model Mech (2012) 1.66
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
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
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
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
Deletion of Atoh1 disrupts Sonic Hedgehog signaling in the developing cerebellum and prevents medulloblastoma. Science (2009) 1.42
Rett syndrome and MeCP2: linking epigenetics and neuronal function. Am J Hum Genet (2002) 1.42
Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis. PLoS Genet (2010) 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
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
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
Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome. Nat Genet (2012) 1.26
Drosophila atonal fully rescues the phenotype of Math1 null mice: new functions evolve in new cellular contexts. Curr Biol (2002) 1.25
Female Mecp2(+/-) mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies. Hum Mol Genet (2012) 1.23
Modelling brain diseases in mice: the challenges of design and analysis. Nat Rev Genet (2003) 1.20
Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxia. Hum Mol Genet (2010) 1.16
SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal. J Biol Chem (2005) 1.15
Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei. J Neurosci (2009) 1.14
ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization. Dev Cell (2011) 1.12
The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7. Proc Natl Acad Sci U S A (2008) 1.11
Dendritic arborization and spine dynamics are abnormal in the mouse model of MECP2 duplication syndrome. J Neurosci (2013) 1.09
Balanced X chromosome inactivation patterns in the Rett syndrome brain. Am J Med Genet (2002) 1.08
Somatic and germline instability of the ATTCT repeat in spinocerebellar ataxia type 10. Am J Hum Genet (2004) 1.08
The role of LANP and ataxin 1 in E4F-mediated transcriptional repression. EMBO Rep (2007) 1.07
Cell-specific expression of wild-type MeCP2 in mouse models of Rett syndrome yields insight about pathogenesis. Hum Mol Genet (2007) 1.06
MeCP2: only 100% will do. Nat Neurosci (2012) 1.05
Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model. Hum Mol Genet (2011) 1.02
Genetic modifiers of MeCP2 function in Drosophila. PLoS Genet (2008) 1.01
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) 1.00
Generation and characterization of LANP/pp32 null mice. Mol Cell Biol (2004) 1.00
In vivo neuronal subtype-specific targets of Atoh1 (Math1) in dorsal spinal cord. J Neurosci (2011) 0.98
Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration. Proc Natl Acad Sci U S A (2011) 0.96
Loss of holocytochrome c-type synthetase causes the male lethality of X-linked dominant microphthalmia with linear skin defects (MLS) syndrome. Hum Mol Genet (2002) 0.95
The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up. J Biol Chem (2004) 0.95
Genetic basis of Rett syndrome. Ment Retard Dev Disabil Res Rev (2002) 0.95
14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus. J Biol Chem (2011) 0.94
Enhanced SUMOylation in polyglutamine diseases. Biochem Biophys Res Commun (2002) 0.92
Atoh1 governs the migration of postmitotic neurons that shape respiratory effectiveness at birth and chemoresponsiveness in adulthood. Neuron (2012) 0.92
The yin and yang of MeCP2 phosphorylation. Proc Natl Acad Sci U S A (2009) 0.91