Adult Ube3a Gene Reinstatement Restores the Electrophysiological Deficits of Prefrontal Cortex Layer 5 Neurons in a Mouse Model of Angelman Syndrome

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Dissociation of locomotor and cerebellar deficits in a murine Angelman syndrome model

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Ubiquitin-dependent endocytosis, trafficking and turnover of neuronal membrane proteins

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Decreased Axon Caliber Underlies Loss of Fiber Tract Integrity, Disproportional Reductions in White Matter Volume, and Microcephaly in Angelman Syndrome Model Mice

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Persistent neuronal Ube3a expression in the suprachiasmatic nucleus of Angelman syndrome model mice

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Ube3a loss increases excitability and blunts orientation tuning in the visual cortex of Angelman syndrome model mice

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Enhanced Transmission at the Calyx of Held Synapse in a Mouse Model for Angelman Syndrome

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The Angelman Syndrome Protein Ube3A Regulates Synapse Development by Ubiquitinating Arc

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The heterogeneity in GABAA receptor-mediated IPSC kinetics reflects heterogeneity of subunit composition among inhibitory and excitatory interneurons in spinal lamina II

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Region‐specific impairments in striatal synaptic transmission and impaired instrumental learning in a mouse model of Angelman syndrome

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From Cortical and Subcortical Grey Matter Abnormalities to Neurobehavioral Phenotype of Angelman Syndrome: A Voxel-Based Morphometry Study

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Protein Expression Dynamics during Postnatal Mouse Brain Development

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Treatment of Cognitive Deficits in Genetic Disorders: A Systematic Review of Clinical Trials of Diet and Drug Treatments

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Different AMPA receptor subtypes mediate the distinct kinetic components of a biphasic EPSC in hippocampal interneurons

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A molecular tightrope

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The sodium channel activator Lu AE98134 normalizes the altered firing properties of fast spiking interneurons in Dlx5/6+/− mice

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Development of prefrontal cortex

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Synaptopathology Involved in Autism Spectrum Disorder

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Cortical interneurons in autism

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Daily Oscillation of the Excitation-Inhibition Balance in Visual Cortical Circuits

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Recapitulation and Reversal of Schizophrenia-Related Phenotypes in Setd1a-Deficient Mice

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Loss of nuclear UBE3A causes electrophysiological and behavioral deficits in mice and is associated with Angelman syndrome

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An ASO therapy for Angelman syndrome that targets an evolutionarily conserved region at the start of the UBE3A-AS transcript

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The Autism and Angelman Syndrome Protein Ube3A/E6AP: The Gene, E3 Ligase Ubiquitination Targets and Neurobiological Functions

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Angelman Syndrome: From Mouse Models to Therapy

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Angelman syndrome: a journey through the brain

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Translational outcomes in a full gene deletion of ubiquitin protein ligase E3A rat model of Angelman syndrome

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RNA interference (RNAi)-based therapeutics for treatment of rare neurologic diseases

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Resolving the Synaptic versus Developmental Dichotomy of Autism Risk Genes

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Genetic backgrounds have unique seizure response profiles and behavioral outcomes following convulsant administration

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Cell-Type Specificity of Genomic Imprinting in Cerebral Cortex

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Loss of nuclear UBE3A activity is the predominant cause of Angelman syndrome in individuals carrying UBE3A missense mutations

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Dosage-sensitive genes in autism spectrum disorders: From neurobiology to therapy

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Antisense oligonucleotide therapy rescues disturbed brain rhythms and sleep in juvenile and adult mouse models of Angelman syndrome

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Human Cerebral Organoids Reveal Early Spatiotemporal Dynamics and Pharmacological Responses of UBE3A

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Molecular mechanisms for targeted ASD treatments

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Visual Sequences Drive Experience-Dependent Plasticity in Mouse Anterior Cingulate Cortex

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Therapeutic strategies for autism: targeting three levels of the central dogma of molecular biology

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Conserved UBE3A subcellular distribution between human and mice is facilitated by non-homologous isoforms

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The role of ubiquitin ligase E3A in polarized contact guidance and rescue strategies in UBE3A-deficient hippocampal neurons

Tonazzini, I., van Woerden, G. M., Masciullo, C., Mientjes, E., Elgersma, Y., & Cecchini, M. G. (2019) · Molecular Autism · doi:10.1186/s13229-019-0293-1 · Cited by 21 · see citing works

Molecular Evolution, Neurodevelopmental Roles and Clinical Significance of HECT-Type UBE3 E3 Ubiquitin Ligases

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Behavioral Evaluation of Angelman Syndrome Mice at Older Ages

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Postnatal Arx transcriptional activity regulates functional properties of PV interneurons

Joseph, D. J., von Deimling, M., Hasegawa, Y., Cristancho, A. G., Ahrens‐Nicklas, R. C., Rogers, S. L., Risbud, R. M., McCoy, A. J., & Marsh, E. D. (2020) · iScience · doi:10.1016/j.isci.2020.101999 · Cited by 19 · see citing works

The Ubiquitinated Axon: Local Control of Axon Development and Function by Ubiquitin

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Autism Spectrum Disorder Genes: Disease-Related Networks and Compensatory Strategies

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Acetate supplementation restores cognitive deficits caused by ARID1A haploinsufficiency in excitatory neurons

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Gene Therapy for Angelman Syndrome: Contemporary Approaches and Future Endeavors

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Identifying the temporal electrophysiological and molecular changes that contribute to TSC-associated epileptogenesis

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Mono-ubiquitination of Rabphilin 3A by UBE3A serves a non-degradative function

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Towards an understanding of Angelman syndrome in mice studies

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Aberrant aggressive behavior in a mouse model of Angelman syndrome

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UBE3A expression during early postnatal brain development is required for proper dorsomedial striatal maturation

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Regional and cellular organization of the autism-associated protein UBE3A/E6AP and its antisense transcript in the brain of the developing rhesus monkey

Ramirez, C. G., Salvador, S. G., Patel, R. K. R., Clark, S., Miller, N. W., James, L. M., Ringelberg, N. W., Simon, J. M., Bennett, J., Amaral, D. G., et al. (2024) · Frontiers in Neuroanatomy · doi:10.3389/fnana.2024.1410791 · Cited by 14 · see citing works

Hyperexcitability and translational phenotypes in a preclinical mouse model of SYNGAP1-related intellectual disability

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Role of Medial Prefrontal Cortical Neurons and Oxytocin Modulation in the Establishment of Social Buffering

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Bioinformatics analyses show dysregulation of calcium-related genes in Angelman syndrome mouse model

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Activity-dependent degradation of Kv4.2 contributes to synaptic plasticity and behavior in Angelman syndrome model mice

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The Role of SUMOylation and Ubiquitination in Brain Ischaemia: Critical Concepts and Clinical Implications

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Adult Camk2a Gene Reinstatement Restores the Learning and Plasticity Deficits of Camk2a Knockout Mice

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Dystonia in Angelman syndrome: a common, unrecognized clinical finding

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Parent perceptions, beliefs, and fears around genetic treatments and cures for children with Angelman syndrome

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Evaluation of a TrkB agonist on spatial and motor learning in the Ube3a mouse model of Angelman syndromePreprint

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Stem cell models of Angelman syndrome

dos Santos, J. C., Appleton, C., Mateus, F. C., Covas, R., Bekman, E., & da Rocha, S. T. (2023) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2023.1274040 · Cited by 9 · see citing works

The Hippocampal Response to Acute Corticosterone Elevation Is Altered in a Mouse Model for Angelman Syndrome

Viho, E. M. G., Punt, A. M., Distel, B., Houtman, R., Kroon, J., Elgersma, Y., & Meijer, O. C. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms24010303 · Cited by 8 · see citing works

Multiscale spatio-temporal dynamics of UBE3A gene in brain physiology and neurodevelopmental disorders

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Study of adhesion and migration dynamics in ubiquitin E3A ligase (UBE3A)-silenced SYSH5Y neuroblastoma cells by micro-structured surfaces

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Electrophysiological Characterization of Regular and Burst Firing Pyramidal Neurons of the Dorsal Subiculum in an Angelman Syndrome Mouse Model

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Postnatal critical-period brain plasticity and neurodevelopmental disorders: revisited circuit mechanisms

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UBE3A stabilization of β-catenin preserves synaptic proteins essential for motor and cognitive functions in Angelman Syndrome

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Recovery of Angelman syndrome rat deficits with UBE3A protein supplementation

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Characterizing spine issues: If offers novel therapeutics to Angelman syndrome

Yang, X. (2020) · Developmental Neurobiology · doi:10.1002/dneu.22757 · Cited by 6 · see citing works

Dissociating motor impairment from five-choice serial reaction time task performance in a mouse model of Angelman syndrome

Negrón-Moreno, P. N., Diep, D. T., Guoynes, C. D., & Sidorov, M. S. (2022) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2022.968159 · Cited by 5 · see citing works

Hyperexcitability and translational phenotypes in a preclinical model of SYNGAP1 mutationsPreprint

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Neural hyperexcitability in Angelman syndrome: Genetic factors and pharmacologic treatment approaches

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Developmental disabilities, autism, and schizophrenia at a single locus

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Angelman syndrome

Mathews, J. M., Cook, E. K., Margolis, S. S., & Williams, C. A. (2020) · Elsevier eBooks · doi:10.1016/b978-0-12-813955-4.00024-6 · Cited by 4 · see citing works

Deficiency of calretinin in prefrontal cortex causes behavioral deficits relevant to autism spectrum disorder in mice

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The Role of SUMOylation and Ubiquitination in Brain Ischaemia: Critical Concepts and Clinical Implications

Bernstock, J. D., Ye, D. G., Estevez, D., Wang, Y., Gessler, F. A., Hallenbeck, J. M., & Yang, W. (2019) · Caister Academic Press eBooks · doi:10.21775/9781912530120.19 · Cited by 2 · see citing works

Periodic and aperiodic contributions to EEG delta power are translatable and complementary Angelman syndrome biomarkers

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UBE3A controls axon initial segment in the cortical pyramidal neurons

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Cortical layer-specific abnormalities in auditory responses in a mouse model of Fragile X Syndrome

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Induced pluripotent stem cells for modeling Angelman syndrome

Maranga, C., Vieira, A. A., Bekman, E., & da Rocha, S. T. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-85764-2.00015-6 · Cited by 1 · see citing works

Hyperexcitability and translational phenotypes in a preclinical model of SYNGAP1 mutationsPreprint

Silverman, J. L., Fenton, T., Haouchine, O. Y., Hallam, E., Smith, E., Jackson, K. C., Rahbarian, D., Canales, C. P., Adhikari, A., Nord, A. S., et al. (2023) · Research Square · doi:10.21203/rs.3.rs-3246655/v1 · Cited by 1 · see citing works

Adult Camk2a Gene Reinstatement Restores the Learning and Plasticity Deficits of Camk2a Knockout MicePreprint

Rigter, P. M. F., Wallaard, I., Jolfaei, M. A., Kingma, J., Post, L. C., Elgersma, M., Elgersma, Y., & van Woerden, G. M. (2022) · SSRN Electronic Journal · doi:10.2139/ssrn.4143785

Angelman syndrome

Mathews, J. M., & Jalazo, E. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-19041-4.00024-8

Investigation of Demographic Characteristics and General Appearance of Individuals with Angelman Syndrome

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The serotonin receptor 7 as an emerging target to restore altered neuroplasticity in Angelman syndrome

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Daily, J. (2012) · Digital Commons - University of South Florida (University of South Florida)