| The role of glia in the dysregulation of neuronal spinogenesis in Ube3a-dependent ASD. | The role of glia in the dysregulation of neuronal spinogenesis in Ube3a-dependent ASD.
Gardner Z, Holbrook O, Tian Y, Odamah K, Man HY., | 05/23/2024 |
| mRNA nuclear retention reduces AMPAR expression and promotes autistic behavior in UBE3A-overexpressing mice. | mRNA nuclear retention reduces AMPAR expression and promotes autistic behavior in UBE3A-overexpressing mice.
Tian Y, Yu F, Yun E, Lin JW, Man HY., Free PMC Article | 03/15/2024 |
| Light activates Ube3a, an Angelman syndrome-associated gene, by mediating the chromatin structures during postnatal development of mouse retina. | Light activates Ube3a, an Angelman syndrome-associated gene, by mediating the chromatin structures during postnatal development of mouse retina.
Lin CW, Cheng YC, Yang CH, Huang HS. | 01/3/2024 |
| UBE3A and MCM6 synergistically regulate the proliferation and migration of lung adenocarcinoma cells. | UBE3A and MCM6 synergistically regulate the proliferation and migration of lung adenocarcinoma cells.
Luo Y, Yang Y, Yang C, Li C, Hu R, Geng W, Kang X, Lin H., Free PMC Article | 09/6/2023 |
| Autism-linked UBE3A gain-of-function mutation causes interneuron and behavioral phenotypes when inherited maternally or paternally in mice. | Autism-linked UBE3A gain-of-function mutation causes interneuron and behavioral phenotypes when inherited maternally or paternally in mice.
Xing L, Simon JM, Ptacek TS, Yi JJ, Loo L, Mao H, Wolter JM, McCoy ES, Paranjape SR, Taylor-Blake B, Zylka MJ., Free PMC Article | 08/3/2023 |
| IL-1beta turnover by the UBE2L3 ubiquitin conjugating enzyme and HECT E3 ligases limits inflammation. | IL-1β turnover by the UBE2L3 ubiquitin conjugating enzyme and HECT E3 ligases limits inflammation.
Mishra V, Crespo-Puig A, McCarthy C, Masonou T, Glegola-Madejska I, Dejoux A, Dow G, Eldridge MJG, Marinelli LH, Meng M, Wang S, Bennison DJ, Morrison R, Shenoy AR., Free PMC Article | 07/27/2023 |
| A high-fidelity RNA-targeting Cas13 restores paternal Ube3a expression and improves motor functions in Angelman syndrome mice. | A high-fidelity RNA-targeting Cas13 restores paternal Ube3a expression and improves motor functions in Angelman syndrome mice.
Li J, Shen Z, Liu Y, Yan Z, Liu Y, Lin X, Tang J, Lv R, Geng G, Xiong ZQ, Zhou C, Yang H., | 07/10/2023 |
| Effects of UBE3A on Cell and Liver Metabolism through the Ubiquitination of PDHA1 and ACAT1. | Effects of UBE3A on Cell and Liver Metabolism through the Ubiquitination of PDHA1 and ACAT1.
Peng K, Wang S, Liu R, Zhou L, Jeong GH, Jeong IH, Liu X, Kiyokawa H, Xue B, Zhao B, Shi H, Yin J., Free PMC Article | 04/10/2023 |
| UBE3A deficiency-induced autophagy is associated with activation of AMPK-ULK1 and p53 pathways. | UBE3A deficiency-induced autophagy is associated with activation of AMPK-ULK1 and p53 pathways.
Hao X, Sun J, Zhong L, Baudry M, Bi X., Free PMC Article | 04/6/2023 |
| UBE3A expression during early postnatal brain development is required for proper dorsomedial striatal maturation. | UBE3A expression during early postnatal brain development is required for proper dorsomedial striatal maturation.
Rotaru DC, Wallaard I, de Vries M, van der Bie J, Elgersma Y., Free PMC Article | 02/24/2023 |
| Lack of UBE3A-Mediated Regulation of Synaptic SK2 Channels Contributes to Learning and Memory Impairment in the Female Mouse Model of Angelman Syndrome. | Lack of UBE3A-Mediated Regulation of Synaptic SK2 Channels Contributes to Learning and Memory Impairment in the Female Mouse Model of Angelman Syndrome.
Sun J, Liu Y, Hao X, Baudry M, Bi X., Free PMC Article | 10/22/2022 |
| Molecular and behavioral consequences of Ube3a gene overdosage in mice. | Molecular and behavioral consequences of Ube3a gene overdosage in mice.
Punt AM, Judson MC, Sidorov MS, Williams BN, Johnson NS, Belder S, den Hertog D, Davis CR, Feygin MS, Lang PF, Jolfaei MA, Curran PJ, van IJcken WF, Elgersma Y, Philpot BD., Free PMC Article | 10/1/2022 |
| Functional rescue in an Angelman syndrome model following treatment with lentivector transduced hematopoietic stem cells. | Functional rescue in an Angelman syndrome model following treatment with lentivector transduced hematopoietic stem cells.
Adhikari A, Copping NA, Beegle J, Cameron DL, Deng P, O'Geen H, Segal DJ, Fink KD, Silverman JL, Anderson JS., Free PMC Article | 03/12/2022 |
| Motor Deficits Coupled to Cerebellar and Striatal Alterations in Ube3a(m-/p+) Mice Modelling Angelman Syndrome Are Attenuated by Adenosine A2A Receptor Blockade. | Motor Deficits Coupled to Cerebellar and Striatal Alterations in Ube3a(m-/p+) Mice Modelling Angelman Syndrome Are Attenuated by Adenosine A(2A) Receptor Blockade.
Moreira-de-Sá A, Gonçalves FQ, Lopes JP, Silva HB, Tomé ÂR, Cunha RA, Canas PM. | 11/27/2021 |
| Evaluation of UBE3A antibodies in mice and human cerebral organoids. | Evaluation of UBE3A antibodies in mice and human cerebral organoids.
Sen D, Drobna Z, Keung AJ., Free PMC Article | 10/16/2021 |
| Conserved UBE3A subcellular distribution between human and mice is facilitated by non-homologous isoforms. | Conserved UBE3A subcellular distribution between human and mice is facilitated by non-homologous isoforms.
Zampeta FI, Sonzogni M, Niggl E, Lendemeijer B, Smeenk H, de Vrij FMS, Kushner SA, Distel B, Elgersma Y., Free PMC Article | 08/28/2021 |
| Abundance and localization of human UBE3A protein isoforms. | Abundance and localization of human UBE3A protein isoforms.
Sirois CL, Bloom JE, Fink JJ, Gorka D, Keller S, Germain ND, Levine ES, Chamberlain SJ., Free PMC Article | 08/28/2021 |
| Assessing the requirements of prenatal UBE3A expression for rescue of behavioral phenotypes in a mouse model for Angelman syndrome. | Assessing the requirements of prenatal UBE3A expression for rescue of behavioral phenotypes in a mouse model for Angelman syndrome.
Sonzogni M, Zhai P, Mientjes EJ, van Woerden GM, Elgersma Y., Free PMC Article | 08/7/2021 |
| Bioinformatics Analyses of the Transcriptome Reveal Ube3a-Dependent Effects on Mitochondrial-Related Pathways. | Bioinformatics Analyses of the Transcriptome Reveal Ube3a-Dependent Effects on Mitochondrial-Related Pathways.
Panov J, Simchi L, Feuermann Y, Kaphzan H., Free PMC Article | 03/27/2021 |
| PKA and Ube3a regulate SK2 channel trafficking to promote synaptic plasticity in hippocampus: Implications for Angelman Syndrome. | PKA and Ube3a regulate SK2 channel trafficking to promote synaptic plasticity in hippocampus: Implications for Angelman Syndrome.
Sun J, Liu Y, Zhu G, Cato C, Hao X, Qian L, Lin W, Adhikari R, Luo Y, Baudry M, Bi X., Free PMC Article | 12/12/2020 |
| our results identify HAP1 as an in vivo UBE3A target that contributes to deregulated autophagy and synaptic dysfunction in the central nervous system of Angelman syndrome mice | HAP1 is an in vivo UBE3A target that augments autophagy in a mouse model of Angelman syndrome.
Wang T, Wang J, Wang J, Mao L, Tang B, Vanderklish PW, Liao X, Xiong ZQ, Liao L. | 08/12/2020 |
| UBE3A-related deficits in early neuronal morphogenesis may lead to defective neuronal connectivity and plasticity. | The role of ubiquitin ligase E3A in polarized contact guidance and rescue strategies in UBE3A-deficient hippocampal neurons.
Tonazzini I, Van Woerden GM, Masciullo C, Mientjes EJ, Elgersma Y, Cecchini M., Free PMC Article | 06/20/2020 |
| UBE3A critically impacts early brain development, but plays a more limited role in adulthood. | Delayed loss of UBE3A reduces the expression of Angelman syndrome-associated phenotypes.
Sonzogni M, Hakonen J, Bernabé Kleijn M, Silva-Santos S, Judson MC, Philpot BD, van Woerden GM, Elgersma Y., Free PMC Article | 06/20/2020 |
| our results place Ube3A as a critical player in Alzheimer's disease pathogenesis, and as a potential therapeutic target. | Dysfunction of the ubiquitin ligase E3A Ube3A/E6-AP contributes to synaptic pathology in Alzheimer's disease.
Olabarria M, Pasini S, Corona C, Robador P, Song C, Patel H, Lefort R., Free PMC Article | 04/18/2020 |
| results implicate a critical role of UBE3A-PTPA-PP2A signaling in the pathogenesis of UBE3A-related disorders and suggest that PP2A-based drugs could be potential therapeutic candidates for treatment of UBE3A-related disorders | UBE3A-mediated PTPA ubiquitination and degradation regulate PP2A activity and dendritic spine morphology.
Wang J, Lou SS, Wang T, Wu RJ, Li G, Zhao M, Lu B, Li YY, Zhang J, Cheng X, Shen Y, Wang X, Zhu ZC, Li MJ, Takumi T, Yang H, Yu X, Liao L, Xiong ZQ., Free PMC Article | 04/11/2020 |