Published in Neuron on October 01, 1998
Physiological functions of the HECT family of ubiquitin ligases. Nat Rev Mol Cell Biol (2009) 5.43
The Angelman Syndrome protein Ube3A regulates synapse development by ubiquitinating arc. Cell (2010) 4.72
Ubiquitination regulates PSD-95 degradation and AMPA receptor surface expression. Neuron (2003) 3.62
Topoisomerase inhibitors unsilence the dormant allele of Ube3a in neurons. Nature (2011) 3.19
Calmodulin-kinases: modulators of neuronal development and plasticity. Neuron (2008) 3.14
Epigenetic overlap in autism-spectrum neurodevelopmental disorders: MECP2 deficiency causes reduced expression of UBE3A and GABRB3. Hum Mol Genet (2004) 2.89
Angelman syndrome: a review of the clinical and genetic aspects. J Med Genet (2003) 2.69
Genome-wide analyses of human perisylvian cerebral cortical patterning. Proc Natl Acad Sci U S A (2007) 2.56
Synaptic dysfunction and abnormal behaviors in mice lacking major isoforms of Shank3. Hum Mol Genet (2011) 2.38
Epigenetic mechanisms in cognition. Neuron (2011) 2.31
Ube3a is required for experience-dependent maturation of the neocortex. Nat Neurosci (2009) 2.28
Neuronal activity-regulated gene transcription in synapse development and cognitive function. Cold Spring Harb Perspect Biol (2011) 2.27
Activity-dependent neuronal signalling and autism spectrum disorder. Nature (2013) 2.23
GABAergic Neuron-Specific Loss of Ube3a Causes Angelman Syndrome-Like EEG Abnormalities and Enhances Seizure Susceptibility. Neuron (2016) 2.14
The comorbidity of autism with the genomic disorders of chromosome 15q11.2-q13. Neurobiol Dis (2008) 1.97
Maternally derived microduplications at 15q11-q13: implication of imprinted genes in psychotic illness. Am J Psychiatry (2011) 1.94
Ubiquitination in postsynaptic function and plasticity. Annu Rev Cell Dev Biol (2010) 1.94
Towards a therapy for Angelman syndrome by targeting a long non-coding RNA. Nature (2014) 1.87
Identification of four highly conserved genes between breakpoint hotspots BP1 and BP2 of the Prader-Willi/Angelman syndromes deletion region that have undergone evolutionary transposition mediated by flanking duplicons. Am J Hum Genet (2003) 1.82
EphB-mediated degradation of the RhoA GEF Ephexin5 relieves a developmental brake on excitatory synapse formation. Cell (2010) 1.82
Reversing neurodevelopmental disorders in adults. Neuron (2008) 1.80
Angelman syndrome: insights into genomic imprinting and neurodevelopmental phenotypes. Trends Neurosci (2011) 1.72
Distinct phenotypes distinguish the molecular classes of Angelman syndrome. J Med Genet (2001) 1.71
Dendritic mRNAs encode diversified functionalities in hippocampal pyramidal neurons. BMC Neurosci (2006) 1.66
Altered activity, social behavior, and spatial memory in mice lacking the NTAN1p amidase and the asparagine branch of the N-end rule pathway. Mol Cell Biol (2000) 1.64
Regulation of Rap2A by the ubiquitin ligase Nedd4-1 controls neurite development. Neuron (2010) 1.57
UBE3A Regulates Synaptic Plasticity and Learning and Memory by Controlling SK2 Channel Endocytosis. Cell Rep (2015) 1.52
Increased gene dosage of Ube3a results in autism traits and decreased glutamate synaptic transmission in mice. Sci Transl Med (2011) 1.48
The corepressor mSin3a interacts with the proline-rich domain of p53 and protects p53 from proteasome-mediated degradation. Mol Cell Biol (2001) 1.47
Ube3a imprinting impairs circadian robustness in Angelman syndrome models. Curr Biol (2015) 1.46
Autism: a "critical period" disorder? Neural Plast (2011) 1.46
Genetics of Angelman syndrome. Am J Hum Genet (1999) 1.43
Targeting the histone methyltransferase G9a activates imprinted genes and improves survival of a mouse model of Prader-Willi syndrome. Nat Med (2016) 1.39
Analysis of cerebellar function in Ube3a-deficient mice reveals novel genotype-specific behaviors. Hum Mol Genet (2008) 1.37
Altered ultrasonic vocalization and impaired learning and memory in Angelman syndrome mouse model with a large maternal deletion from Ube3a to Gabrb3. PLoS One (2010) 1.37
Tissue-specific variation of Ube3a protein expression in rodents and in a mouse model of Angelman syndrome. Neurobiol Dis (2010) 1.33
Alterations in intrinsic membrane properties and the axon initial segment in a mouse model of Angelman syndrome. J Neurosci (2011) 1.31
Ras and Rap signaling in synaptic plasticity and mental disorders. Neuroscientist (2010) 1.29
Degradation of postsynaptic scaffold GKAP and regulation of dendritic spine morphology by the TRIM3 ubiquitin ligase in rat hippocampal neurons. PLoS One (2010) 1.28
The emerging field of neuroepigenetics. Neuron (2013) 1.28
Molecular and Clinical Aspects of Angelman Syndrome. Mol Syndromol (2011) 1.27
Genomic imprinting of experience-dependent cortical plasticity by the ubiquitin ligase gene Ube3a. Proc Natl Acad Sci U S A (2010) 1.27
Expression of the Rho-GEF Pbl/ECT2 is regulated by the UBE3A E3 ubiquitin ligase. Hum Mol Genet (2006) 1.25
Imprinted Nesp55 influences behavioral reactivity to novel environments. Mol Cell Biol (2005) 1.25
Association of E6AP (UBE3A) with human papillomavirus type 11 E6 protein. Virology (2006) 1.22
A Drosophila model for Angelman syndrome. Proc Natl Acad Sci U S A (2008) 1.21
Molecular mechanism of angelman syndrome in two large families involves an imprinting mutation. Am J Hum Genet (1999) 1.21
The ubiquitin-proteasome pathway and synaptic plasticity. Learn Mem (2010) 1.20
HECT E3s and human disease. BMC Biochem (2007) 1.19
Characterization of the Brain 26S Proteasome and its Interacting Proteins. Front Mol Neurosci (2010) 1.19
A transgene insertion creating a heritable chromosome deletion mouse model of Prader-Willi and angelman syndromes. Proc Natl Acad Sci U S A (1999) 1.19
Truncation of Ube3a-ATS unsilences paternal Ube3a and ameliorates behavioral defects in the Angelman syndrome mouse model. PLoS Genet (2013) 1.18
Cognitive neuroepigenetics: a role for epigenetic mechanisms in learning and memory. Neurobiol Learn Mem (2010) 1.16
Behavioral deficits in an Angelman syndrome model: effects of genetic background and age. Behav Brain Res (2013) 1.15
Angelman syndrome: review of clinical and molecular aspects. Appl Clin Genet (2014) 1.14
Ca(2+)/calmodulin-dependent protein kinase IIα (αCaMKII) controls the activity of the dopamine transporter: implications for Angelman syndrome. J Biol Chem (2012) 1.14
Impairment of TrkB-PSD-95 signaling in Angelman syndrome. PLoS Biol (2013) 1.13
Spontaneous seizures and altered gene expression in GABA signaling pathways in a mind bomb mutant zebrafish. J Neurosci (2010) 1.13
Maternal loss of Ube3a produces an excitatory/inhibitory imbalance through neuron type-specific synaptic defects. Neuron (2012) 1.12
Arc/Arg3.1 is a postsynaptic mediator of activity-dependent synapse elimination in the developing cerebellum. Neuron (2013) 1.10
Ampakines promote spine actin polymerization, long-term potentiation, and learning in a mouse model of Angelman syndrome. Neurobiol Dis (2012) 1.08
HPV E6, E6AP and cervical cancer. BMC Biochem (2008) 1.08
The Drosophila homologue of the Angelman syndrome ubiquitin ligase regulates the formation of terminal dendritic branches. Hum Mol Genet (2008) 1.08
Genomic imprinting and the social brain. Philos Trans R Soc Lond B Biol Sci (2006) 1.08
Allelic specificity of Ube3a expression in the mouse brain during postnatal development. J Comp Neurol (2014) 1.07
Induced pluripotent stem (iPS) cells as in vitro models of human neurogenetic disorders. Neurogenetics (2008) 1.06
Activity-dependent phosphorylation of GABAA receptors regulates receptor insertion and tonic current. EMBO J (2012) 1.06
Imbalanced mechanistic target of rapamycin C1 and C2 activity in the cerebellum of Angelman syndrome mice impairs motor function. J Neurosci (2015) 1.06
E6-associated protein (E6-AP) is a dual function coactivator of steroid hormone receptors. Nucl Recept Signal (2008) 1.04
Reversal of impaired hippocampal long-term potentiation and contextual fear memory deficits in Angelman syndrome model mice by ErbB inhibitors. Biol Psychiatry (2012) 1.02
Role of ubiquitin-proteasome-mediated proteolysis in nervous system disease. Biochim Biophys Acta (2010) 1.01
The deubiquitinating enzyme USP-46 negatively regulates the degradation of glutamate receptors to control their abundance in the ventral nerve cord of Caenorhabditis elegans. J Neurosci (2011) 1.01
Gonadal transcriptome alterations in response to dietary energy intake: sensing the reproductive environment. PLoS One (2009) 1.00
HPV16 E6 confers p53-dependent and p53-independent phenotypes in the epidermis of mice deficient for E6AP. Oncogene (2006) 1.00
The role of GNAS and other imprinted genes in the development of obesity. Int J Obes (Lond) (2009) 1.00
Epigenetics and Neural developmental disorders: Washington DC, September 18 and 19, 2006. Epigenetics (2007) 0.99
Interactions between imprinting effects in the mouse. Genetics (2004) 0.99
Role of the ubiquitin ligase E6AP/UBE3A in controlling levels of the synaptic protein Arc. Proc Natl Acad Sci U S A (2013) 0.99
The ubiquitin-proteasome system is necessary for long-term synaptic depression in Aplysia. J Neurosci (2008) 0.98
Normal social seeking behavior, hypoactivity and reduced exploratory range in a mouse model of Angelman syndrome. BMC Genet (2011) 0.98
Physical and functional interaction of the HECT ubiquitin-protein ligases E6AP and HERC2. J Biol Chem (2011) 0.98
p53 and mitochondrial function in neurons. Biochim Biophys Acta (2014) 0.97
Maternal disruption of Ube3a leads to increased expression of Ube3a-ATS in trans. Nucleic Acids Res (2005) 0.97
Protein degradation and memory formation. Brain Res Bull (2010) 0.96
Double-blind therapeutic trial in Angelman syndrome using betaine and folic acid. Am J Med Genet A (2010) 0.96
Genetic ablation of the steroid receptor coactivator-ubiquitin ligase, E6-AP, results in tissue-selective steroid hormone resistance and defects in reproduction. Mol Cell Biol (2002) 0.95
Adeno-associated virus-mediated rescue of the cognitive defects in a mouse model for Angelman syndrome. PLoS One (2011) 0.95
Genetic reduction of the α1 subunit of Na/K-ATPase corrects multiple hippocampal phenotypes in Angelman syndrome. Cell Rep (2013) 0.94
Prader-Willi, Angelman, and 15q11-q13 Duplication Syndromes. Pediatr Clin North Am (2015) 0.94
Ube3a reinstatement identifies distinct developmental windows in a murine Angelman syndrome model. J Clin Invest (2015) 0.94
A therapeutic trial of pro-methylation dietary supplements in Angelman syndrome. Am J Med Genet A (2011) 0.94
The role of deubiquitinating enzymes in synaptic function and nervous system diseases. Neural Plast (2012) 0.93
Behavior and neuropsychiatric manifestations in Angelman syndrome. Neuropsychiatr Dis Treat (2008) 0.92
Molecular mechanisms involved in progesterone receptor regulation of uterine function. J Steroid Biochem Mol Biol (2006) 0.92
Fruit flies and intellectual disability. Fly (Austin) (2009) 0.92
Genetic control of postnatal human brain growth. Curr Opin Neurol (2016) 0.91
Mitochondrial dysfunction in CA1 hippocampal neurons of the UBE3A deficient mouse model for Angelman syndrome. Neurosci Lett (2009) 0.91
An Autism-Linked Mutation Disables Phosphorylation Control of UBE3A. Cell (2015) 0.91
Epigenetic regulation of UBE3A and roles in human neurodevelopmental disorders. Epigenomics (2015) 0.90
E6-associated protein is required for human papillomavirus type 16 E6 to cause cervical cancer in mice. Cancer Res (2010) 0.90
Embryonic lethality and radiation hypersensitivity mediated by Rad51 in mice lacking Brca2. Nature (1997) 8.04
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat Genet (1993) 7.97
Endoscopic assessment of oesophagitis: clinical and functional correlates and further validation of the Los Angeles classification. Gut (1999) 7.14
Mycobacterium avium complex infections in patients with the acquired immunodeficiency syndrome. Ann Intern Med (1986) 6.79
9-cis retinoic acid is a high affinity ligand for the retinoid X receptor. Cell (1992) 6.75
Effect of a general practitioner's consulting style on patients' satisfaction: a controlled study. BMJ (1990) 6.15
p53-independent expression of p21Cip1 in muscle and other terminally differentiating cells. Science (1995) 6.01
Infections caused by Mycobacterium avium complex in immunocompromised patients: diagnosis by blood culture and fecal examination, antimicrobial susceptibility tests, and morphological and seroagglutination characteristics. J Clin Microbiol (1985) 5.22
Referral to hospital: perceptions of patients, general practitioners and consultants about necessity and suitability of referral. Fam Pract (1987) 5.11
The mPer2 gene encodes a functional component of the mammalian circadian clock. Nature (1999) 4.74
Expression of vascular endothelial growth factor during embryonic angiogenesis and endothelial cell differentiation. Development (1992) 4.69
Requirement for Wnt3 in vertebrate axis formation. Nat Genet (1999) 4.68
De novo truncating mutations in E6-AP ubiquitin-protein ligase gene (UBE3A) in Angelman syndrome. Nat Genet (1997) 4.65
A steroid receptor coactivator, SRA, functions as an RNA and is present in an SRC-1 complex. Cell (1999) 4.65
The MAPK cascade is required for mammalian associative learning. Nat Neurosci (1998) 4.61
Nonredundant roles of the mPer1 and mPer2 genes in the mammalian circadian clock. Cell (2001) 4.61
Mutation analysis for heterozygote detection and the prenatal diagnosis of cystic fibrosis. N Engl J Med (1990) 4.60
Comparison of criteria derived by government and patients for evaluating general practitioner services. BMJ (1989) 4.36
Fungemia in the immunocompromised host. Changing patterns, antigenemia, high mortality. Am J Med (1981) 4.31
Uniparental disomy as a mechanism for human genetic disease. Am J Hum Genet (1988) 4.21
A differential response of two putative mammalian circadian regulators, mper1 and mper2, to light. Cell (1997) 4.05
Deletions of a differentially methylated CpG island at the SNRPN gene define a putative imprinting control region. Nat Genet (1994) 4.04
Treatment of infections in patients with the acquired immunodeficiency syndrome. Ann Intern Med (1985) 4.02
Fungemia in a cancer hospital: changing frequency, earlier onset, and results of therapy. Rev Infect Dis (1985) 4.00
Sequential contribution of L- and P-selectin to leukocyte rolling in vivo. J Exp Med (1995) 3.83
Aspergillosis complicating neoplastic disease. Am J Med (1973) 3.80
Diagnostic criteria for Walker-Warburg syndrome. Am J Med Genet (1989) 3.74
Implementing evidence based medicine in general practice: audit and qualitative study of antithrombotic treatment for atrial fibrillation. BMJ (1999) 3.62
Inflammatory and immune responses are impaired in mice deficient in intercellular adhesion molecule 1. Proc Natl Acad Sci U S A (1993) 3.57
Identification and spatial distribution of retinoids in the developing chick limb bud. Nature (1987) 3.49
An evidence-based approach to the management of uninvestigated dyspepsia in the era of Helicobacter pylori. Canadian Dyspepsia Working Group. CMAJ (2000) 3.45
Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons. Nat Genet (1997) 3.32
Human immunity to Pseudomonas aeruginosa. I. In-vitro interaction of bacteria, polymorphonuclear leukocytes, and serum factors. J Infect Dis (1972) 3.28
Megestrol acetate in patients with AIDS-related cachexia. Ann Intern Med (1994) 3.25
RIGUI, a putative mammalian ortholog of the Drosophila period gene. Cell (1997) 3.24
Continuous high-grade mycobacterium avium-intracellulare bacteremia in patients with the acquired immune deficiency syndrome. Am J Med (1985) 3.19
Reasons for referral to hospital: extent of agreement between the perceptions of patients, general practitioners and consultants. Fam Pract (1986) 3.14
Comparative recovery of bacteria and yeasts from lysis-centrifugation and a conventional blood culture system. J Clin Microbiol (1983) 3.09
The American Society of Human Genetics statement on cystic fibrosis screening. Am J Hum Genet (1990) 3.07
A requirement for the mitogen-activated protein kinase cascade in hippocampal long term potentiation. J Biol Chem (1997) 3.06
Activity of voriconazole (UK-109,496) against clinical isolates of Aspergillus species and its effectiveness in an experimental model of invasive pulmonary aspergillosis. Antimicrob Agents Chemother (1997) 3.06
Infectious susceptibility and severe deficiency of leukocyte rolling and recruitment in E-selectin and P-selectin double mutant mice. J Exp Med (1996) 3.04
A reevaluation of cancer incidence near the Three Mile Island nuclear plant: the collision of evidence and assumptions. Environ Health Perspect (1997) 3.04
Investigation of burnout in a sample of British general practitioners. Br J Gen Pract (1995) 3.03
Intraorbital wood. Detection by magnetic resonance imaging. Ophthalmology (1990) 2.99
Bacteremia and fungemia complicating neoplastic disease. A study of 364 cases. Am J Med (1977) 2.92
Measuring general practitioner referrals: patient, workload and list size effects. J R Coll Gen Pract (1988) 2.91
Mice deficient in the alpha7 neuronal nicotinic acetylcholine receptor lack alpha-bungarotoxin binding sites and hippocampal fast nicotinic currents. J Neurosci (1997) 2.87
The prevalence of clinically significant endoscopic findings in primary care patients with uninvestigated dyspepsia: the Canadian Adult Dyspepsia Empiric Treatment - Prompt Endoscopy (CADET-PE) study. Aliment Pharmacol Ther (2003) 2.83
Spontaneous skin ulceration and defective T cell function in CD18 null mice. J Exp Med (1998) 2.81
Bronchoalveolar lavage in the diagnosis of diffuse pulmonary infiltrates in the immunosuppressed host. Ann Intern Med (1984) 2.80
Mutation in AP-3 delta in the mocha mouse links endosomal transport to storage deficiency in platelets, melanosomes, and synaptic vesicles. Neuron (1998) 2.73
Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol (1999) 2.73
LFA-1 is sufficient in mediating neutrophil emigration in Mac-1-deficient mice. J Clin Invest (1997) 2.71
8-Azaguanine resistance in mammalian cells. I. Hypoxanthine-guanine phosphoribosyltransferase. Genetics (1972) 2.71
Species identification of coagulase-negative staphylococcal isolates from blood cultures. J Clin Microbiol (1982) 2.71
Preferences of healthy and ill patients for style of general practitioner care: implications for workload and financial incentives under the new contract. Br J Gen Pract (1991) 2.66
Genomic DNA transfer with a high-capacity adenovirus vector results in improved in vivo gene expression and decreased toxicity. Nat Genet (1998) 2.65
Activation of ERK/MAP kinase in the amygdala is required for memory consolidation of pavlovian fear conditioning. J Neurosci (2000) 2.64
Assembling a clock for all seasons: are there M and E oscillators in the genes? J Biol Rhythms (2001) 2.62
Telephone consultations in general practice: an additional or alternative service? Br J Gen Pract (1995) 2.62
Tobramycin (nebramycin factor 6): in vitro activity against Pseudomonas aeruginosa. Appl Microbiol (1971) 2.60
Large expansion of the ATTCT pentanucleotide repeat in spinocerebellar ataxia type 10. Nat Genet (2000) 2.60
Septicemia due to Aeromonas hydrophila: clinical and immunologic aspects. J Infect Dis (1973) 2.55
Decreased resistance to bacterial infection and granulocyte defects in IAP-deficient mice. Science (1996) 2.55
Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease. J Biol Chem (1975) 2.55
Oxidative damage to DNA in diabetes mellitus. Lancet (1996) 2.54
Serologic responses to Pneumocystis carinii antigens in health and disease. J Infect Dis (1990) 2.51
Patients' views of priority setting in health care: an interview survey in one practice. BMJ (1995) 2.50
Patients' responses to risk information about the benefits of treating hypertension. Br J Gen Pract (2001) 2.50
Voltage-activated calcium channels that must be phosphorylated to respond to membrane depolarization. Proc Natl Acad Sci U S A (1987) 2.48
Administration of helper-dependent adenoviral vectors and sequential delivery of different vector serotype for long-term liver-directed gene transfer in baboons. Proc Natl Acad Sci U S A (1999) 2.41
Opportunistic infection in previously healthy women. Initial manifestations of a community-acquired cellular immunodeficiency. Ann Intern Med (1982) 2.41
From the Infectious Diseases Society of America. Guidelines for the use of antimicrobial agents in neutropenic patients with unexplained fever. J Infect Dis (1990) 2.39
Tuberculosis complicating neoplastic disease. A review of 201 cases. Cancer (1974) 2.36
P-Selectin or intercellular adhesion molecule (ICAM)-1 deficiency substantially protects against atherosclerosis in apolipoprotein E-deficient mice. J Exp Med (2000) 2.32
Gene targeting yields a CD18-mutant mouse for study of inflammation. J Immunol (1993) 2.32
Loss of BETA2/NeuroD leads to malformation of the dentate gyrus and epilepsy. Proc Natl Acad Sci U S A (2000) 2.32
Pneumothorax in AIDS. Ann Intern Med (1991) 2.29
Transit time of leukocytes rolling through venules controls cytokine-induced inflammatory cell recruitment in vivo. J Clin Invest (1998) 2.28
Candidiasis: detection by gas-liquid chromatography of D-arabinitol, a fungal metabolite, in human serum. Science (1979) 2.26
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron (1999) 2.25
Activation of p42 mitogen-activated protein kinase in hippocampal long term potentiation. J Biol Chem (1996) 2.24
Nocardia asteroides infection complicating neoplastic disease. Am J Med (1971) 2.23
Infectious morbidity associated with long-term use of venous access devices in patients with cancer. Ann Intern Med (1993) 2.22
The prevalence of yeasts in clinical specimens from cancer patients. Am J Clin Pathol (1980) 2.19
Functional conservation of atonal and Math1 in the CNS and PNS. Development (2000) 2.18
Phycomycosis complicating leukemia and lymphoma. Ann Intern Med (1972) 2.17
Increased arabinitol levels in experimental candidiasis in rats: arabinitol appearance rates, arabinitol/creatinine ratios, and severity of infection. J Infect Dis (1982) 2.16
Nude mouse: a new experimental model for Pneumocystis carinii infection. Science (1977) 2.16
Beta-amyloid activates the mitogen-activated protein kinase cascade via hippocampal alpha7 nicotinic acetylcholine receptors: In vitro and in vivo mechanisms related to Alzheimer's disease. J Neurosci (2001) 2.15
Mechanism of action of aspartate aminotransferase proposed on the basis of its spatial structure. J Mol Biol (1984) 2.15
1997 guidelines for the use of antimicrobial agents in neutropenic patients with unexplained fever. Infectious Diseases Society of America. Clin Infect Dis (1997) 2.14