Loss of dopaminergic neurons and resulting behavioural deficits in mouse model of Angelman syndrome

The HPV-16 E6 and E6-AP complex functions as a ubiquitin-protein ligase in the ubiquitination of p53

Scheffner, M., Huibregtse, J. M., Vierstra, R. D., & Howley, P. M. (1993) · Cell · doi:10.1016/0092-8674(93)90384-3 · Cited by 2,387 · see citing works

UBE3A/E6-AP mutations cause Angelman syndrome

Kishino, T., Lalande, M., & Wagstaff, J. E. (1997) · Nature Genetics · doi:10.1038/ng0197-70 · Cited by 1,304 · see citing works

Parkin-deficient Mice Exhibit Nigrostriatal Deficits but Not Loss of Dopaminergic Neurons

Goldberg, M. S., Fleming, S. M., Palacino, J. J., Cepeda, C., Lam, H. A., Bhatnagar, A., Meloni, E. G., Wu, N., Ackerson, L. C., Klapstein, G. J., et al. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m308947200 · Cited by 913 · see citing works

Mutation of the Angelman Ubiquitin Ligase in Mice Causes Increased Cytoplasmic p53 and Deficits of Contextual Learning and Long-Term Potentiation

Jiang, Y.-H., Armstrong, D., Albrecht, U., Atkins, C. M., Noebels, J. L., Eichele, G., Sweatt, J. D., & Beaudet, A. L. (1998) · Neuron · doi:10.1016/s0896-6273(00)80596-6 · Cited by 852 · see citing works

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

Tanaka, M., Machida, Y., Niu, S., Ikeda, T., Jana, N. R., Doi, H., Kurosawa, M., Nekooki, M., & Nukina, N. (2004) · Nature Medicine · doi:10.1038/nm985 · Cited by 777 · see citing works

Hypersensitivity of DJ-1-deficient mice to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyrindine (MPTP) and oxidative stress

Kim, R. H., Smith, P. D., Aleyasin, H., Hayley, S. P., Mount, M. P., Pownall, S., Wakeham, A. C., You-Ten, A. J., Kalia, S. K., Horne, P., et al. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0501282102 · Cited by 697 · see citing works

Angelman syndrome: a review of the clinical and genetic aspects

Clayton-Smith, J., & Laan, L. (2003) · Journal of Medical Genetics · doi:10.1136/jmg.40.2.87 · Cited by 687 · see citing works

Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons

Albrecht, U., Sutcliffe, J. S., Cattanach, B. M., BEECHEY, C. V., Armstrong, D. L., Gregor Eichele, C., & Beaudet, A. L. (1997) · Nature Genetics · doi:10.1038/ng0997-75 · Cited by 586 · see citing works

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

Cummings, C., Reinstein, E., Sun, Y., Antalffy, B. A., Jiang, Y.-H., Ciechanover, A., Orr, H., Beaudet, A., & Zoghbi, H. (1999) · Neuron · doi:10.1016/s0896-6273(00)81035-1 · Cited by 513 · see citing works

Early and Progressive Sensorimotor Anomalies in Mice Overexpressing Wild-Type Human α-Synuclein

Fleming, S. M., Salcedo, J. D., Fernagut, P., Rockenstein, E. M., Masliah, E., Levine, M. S., & Chesselet, M. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.3080-04.2004 · Cited by 477 · see citing works

Pole test is a useful method for evaluating the mouse movement disorder caused by striatal dopamine depletion

Matsuura, K., Kabuto, H., Makino, H., & Ogawa, N. (1997) · Journal of Neuroscience Methods · doi:10.1016/s0165-0270(96)02211-x · Cited by 423 · see citing works

The Angelman syndrome ubiquitin ligase localizes to the synapse and nucleus, and maternal deficiency results in abnormal dendritic spine morphology

Dindot, S. V., Antalffy, B. A., Bhattacharjee, M. B., & Beaudet, A. L. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm288 · Cited by 418 · see citing works

The Angelman syndrome candidate gene, UBE3AIE6-AP, is imprinted in brain

Rougeulle, C., Glatt, H., & Lalande, M. (1997) · Nature Genetics · doi:10.1038/ng0997-14 · Cited by 416 · see citing works

Ube3a is required for experience-dependent maturation of the neocortex

Yashiro, K., Riday, T. T., Condon, K. H., Roberts, A. C., Bernardo, D. R., Prakash, R., Weinberg, R. J., Ehlers, M. D., & Philpot, B. D. (2009) · Nature Neuroscience · doi:10.1038/nn.2327 · Cited by 328 · see citing works

Derangements of Hippocampal Calcium/Calmodulin-Dependent Protein Kinase II in a Mouse Model for Angelman Mental Retardation Syndrome

Weeber, E. J., Jiang, Y., Elgersma, Y., Varga, A. W., Carrasquillo, Y., Brown, S. E., Christian, J. M., Mirnikjoo, B., Silva, A. J., Beaudet, A. L., et al. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-07-02634.2003 · Cited by 262 · see citing works

Behavioral models of Parkinson's disease in rodents: A new look at an old problem

Meredith, G. E., & Kang, U. J. (2006) · Movement Disorders · doi:10.1002/mds.21010 · Cited by 255 · see citing works

The spectrum of mutations in UBE3A causing Angelman syndrome

Fang, P. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.1.129 · Cited by 159 · see citing works

Abnormal Ca2+-Calmodulin-Dependent Protein Kinase II Function Mediates Synaptic and Motor Deficits in Experimental Parkinsonism

Picconi, B. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.1224-04.2004 · Cited by 156 · see citing works

Activation of apoptosis signal regulating kinase 1 (ASK1) and translocation of death‐associated protein, Daxx, in substantia nigra pars compacta in a mouse model of Parkinson's disease: protection by α‐lipoic acid

Karunakaran, S., Diwakar, L., Saeed, U., Agarwal, V., Ramakrishnan, S., Iyengar, S., & Ravindranath, V. (2007) · The FASEB Journal · doi:10.1096/fj.06-7580com · Cited by 140 · see citing works

Different HECT domain ubiquitin ligases employ distinct mechanisms of polyubiquitin chain synthesis

Wang, M., & Pickart, C. M. (2005) · The EMBO Journal · doi:10.1038/sj.emboj.7600895 · Cited by 134 · see citing works

Analysis of cerebellar function in Ube3a-deficient mice reveals novel genotype-specific behaviors

Heck, D. H., Zhao, Y., Roy, S., LeDoux, M. S., & Reiter, L. T. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn117 · Cited by 124 · see citing works

Progressive behavioral deficits in DJ-1-deficient mice are associated with normal nigrostriatal function

Chandran, J. S., Lin, X. Z., Zapata, A. G., Höke, A., Shimoji, M., Moore, S. O., Galloway, M. P., Laird, F. M., Wong, P. C., Price, D. L., et al. (2007) · Neurobiology of Disease · doi:10.1016/j.nbd.2007.11.011 · Cited by 112 · see citing works

Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington’s disease

McBride, J. L., Ramaswamy, S., Gasmi, M., Bartus, R. T., Herzog, C. D., Brandon, E. P., Zhou, L., Pitzer, M. R., Berry‐Kravis, E., & Kordower, J. H. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0508875103 · Cited by 106 · see citing works

Tissue-specific variation of Ube3a protein expression in rodents and in a mouse model of Angelman syndrome

Gustin, R. M., Bichell, T. J., Bubser, M., Daily, J., Filonova, I., Mrelashvili, D., Deutch, A. Y., Colbran, R. J., Weeber, E. J., & Haas, K. F. (2010) · Neurobiology of Disease · doi:10.1016/j.nbd.2010.04.012 · Cited by 105 · see citing works

The Ubiquitin Ligase E6-AP Is Induced and Recruited to Aggresomes in Response to Proteasome Inhibition and May Be Involved in the Ubiquitination of Hsp70-bound Misfolded Proteins

Mishra, A., Godavarthi, S. K., Maheshwari, M., Goswami, A., & Jana, N. R. (2009) · Journal of Biological Chemistry · doi:10.1074/jbc.m806804200 · Cited by 99 · see citing works

E6-AP Promotes Misfolded Polyglutamine Proteins for Proteasomal Degradation and Suppresses Polyglutamine Protein Aggregation and Toxicity

Mishra, A., Dikshit, P., Purkayastha, S., Sharma, J., Nukina, N., & Jana, N. R. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m706620200 · Cited by 94 · see citing works

Dopamine depletion alters phosphorylation of striatal proteins in a model of Parkinsonism

Brown, A. M., Deutch, A. Y., & Colbran, R. J. (2005) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2005.04190.x · Cited by 93 · see citing works

A new motor test sensitive to aging and dopaminergic function

Drucker-Colı́n, R., & García-Hernández, F. (1991) · Journal of Neuroscience Methods · doi:10.1016/0165-0270(91)90081-a · Cited by 64 · see citing works

The ubiquitin ligase E6‐AP promotes degradation of α‐synuclein

Mulherkar, S. A., Sharma, J., & Jana, N. R. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06293.x · Cited by 60 · see citing works

Regulation of turnover of tumor suppressor p53 and cell growth by E6-AP, a ubiquitin protein ligase mutated in Angelman mental retardation syndrome

Mishra, A., & Jana, N. R. (2008) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-007-7476-1 · Cited by 47 · see citing works

Levodopa responsive Parkinsonism in adults with Angelman Syndrome

Harbord, M. G. (2001) · Journal of Clinical Neuroscience · doi:10.1054/jocn.2000.0753 · Cited by 44 · see citing works

A beam-walking apparatus to assess behavioural impairments in MPTP-treated mice: Pharmacological validation with R-(−)-deprenyl

Quinn, L. P., Perren, M. J., Brackenborough, K. T., Woodhams, P. L., Vidgeon-Hart, M., Chapman, H., Pangalos, M. N., Upton, N., & Virley, D. J. (2007) · Journal of Neuroscience Methods · doi:10.1016/j.jneumeth.2007.03.021 · Cited by 40 · see citing works

Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings

Dichter, G. S., Damiano, C. A., & Allen, J. A. (2012) · Journal of Neurodevelopmental Disorders · doi:10.1186/1866-1955-4-19 · Cited by 299 · see citing works

Angelman syndrome: insights into genomic imprinting and neurodevelopmental phenotypes

Mabb, A. M., Judson, M. C., Zylka, M. J., & Philpot, B. D. (2011) · Trends in Neurosciences · doi:10.1016/j.tins.2011.04.001 · Cited by 263 · see citing works

Protein homeostasis and aging: Role of ubiquitin protein ligases

Jana, N. R. (2012) · Neurochemistry International · doi:10.1016/j.neuint.2012.02.009 · Cited by 67 · see citing works

Drosophila Ube3a regulates monoamine synthesis by increasing GTP cyclohydrolase I activity via a non-ubiquitin ligase mechanism

Ferdousy, F., Bodeen, W., Summers, K., Doherty, O., Wright, O., Elsisi, N., Hilliard, G., O'Donnell, J. M., & Reiter, L. T. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2010.12.001 · Cited by 43 · see citing works

Development of an ASO therapy for Angelman syndrome by targeting an evolutionarily conserved region at the start of the UBE3A-AS transcriptPreprint

Dindot, S. V., Christian, S., Murphy, W. J., et al. (2021) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2021.07.27.453820 · Cited by 2 · see citing works

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

Lee, D., Chen, W., Kaku, H. N., et al. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.06.18.496687 · Cited by 3 · see citing works

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

Lee, D., Chen, W., Kaku, H. N., Zhuo, X., Chao, E. S., Soriano, A., Kuncheria, A., Flores, S., Kim, J. H., Rivera, A. M., et al. (2023) · eLife · doi:10.7554/elife.81892 · Cited by 36 · see citing works

UBE3A reinstatement as a disease‐modifying therapy for Angelman syndrome

Elgersma, Y., & Sonzogni, M. (2021) · Developmental Medicine & Child Neurology · doi:10.1111/dmcn.14831 · Cited by 55 · see citing works

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

(2022) · eLife · doi:10.7554/elife.81892.sa1