A comprehensive atlas of E3 ubiquitin ligase mutations in neurological disorders

AJ George, YC Hoffiz, AJ Charles, Y Zhu… - Frontiers in …, 2018‏ - frontiersin.org
Protein ubiquitination is a posttranslational modification that plays an integral part in
mediating diverse cellular functions. The process of protein ubiquitination requires an …

Angelman syndrome: review of clinical and molecular aspects

LM Bird - The application of clinical genetics, 2014‏ - Taylor & Francis
“Angelman syndrome”(AS) is a neurodevelopmental disorder whose main features are
intellectual disability, lack of speech, seizures, and a characteristic behavioral profile. The …

Neurologic manifestations of Angelman syndrome

RL Thibert, AM Larson, DT Hsieh, AR Raby… - Pediatric neurology, 2013‏ - Elsevier
Angelman syndrome is a neurogenetic disorder characterized by the loss or reduction of the
ubiquitin-protein ligase E3A enzyme. Angelman syndrome results from a deletion or …

GABAergic neuron-specific loss of Ube3a causes Angelman syndrome-like EEG abnormalities and enhances seizure susceptibility

MC Judson, ML Wallace, MS Sidorov, AC Burette, B Gu… - Neuron, 2016‏ - cell.com
Loss of maternal UBE3A causes Angelman syndrome (AS), a neurodevelopmental disorder
associated with severe epilepsy. We previously implicated GABAergic deficits onto layer (L) …

Epilepsy in Angelman syndrome: a sco** review

D Samanta - Brain and Development, 2021‏ - Elsevier
Angelman Syndrome (AS) is characterized by severe developmental delays including
marked speech impairment, movement abnormalities (ataxia, tremor), and unique behaviors …

[HTML][HTML] Angelman syndrome: from mouse models to therapy

DC Rotaru, EJ Mientjes, Y Elgersma - Neuroscience, 2020‏ - Elsevier
The UBE3A gene is part of the chromosome 15q11-q13 region that is frequently deleted or
duplicated, leading to several neurodevelopmental disorders (NDD). Angelman syndrome …

A multidisciplinary approach and consensus statement to establish standards of care for Angelman syndrome

J Duis, M Nespeca, J Summers, L Bird… - … Genetics & Genomic …, 2022‏ - Wiley Online Library
Background Angelman syndrome (AS) is a rare neurogenetic disorder present in
approximately 1/12,000 individuals and characterized by developmental delay, cognitive …

Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis

MS Sidorov, GM Deck, M Dolatshahi, RL Thibert… - Journal of …, 2017‏ - Springer
Background Clinicians have qualitatively described rhythmic delta activity as a prominent
EEG abnormality in individuals with Angelman syndrome, but this phenotype has yet to be …

[HTML][HTML] Electrophysiological phenotype in Angelman syndrome differs between genotypes

J Frohlich, MT Miller, LM Bird, P Garces, H Purtell… - Biological …, 2019‏ - Elsevier
Background Angelman syndrome (AS) is a severe neurodevelopmental disorder caused by
either disruptions of the gene UBE3A or deletion of chromosome 15 at 15q11-q13, which …

[HTML][HTML] Genotype–phenotype correlations in Angelman syndrome

L Yang, X Shu, S Mao, Y Wang, X Du, C Zou - Genes, 2021‏ - mdpi.com
Angelman syndrome (AS) is a rare neurodevelopmental disease that is caused by the loss
of function of the maternal copy of ubiquitin–protein ligase E3A (UBE3A) on the …