Peptidomimetic inhibitors targeting TrkB/PSD-95 signaling improves cognition and seizure outcomes in an Angelman Syndrome mouse model

EZ Huie, X Yang, MS Rioult-Pedotti, MT Naik… - bioRxiv, 2024 - biorxiv.org
Angelman syndrome (AS) is a rare genetic neurodevelopmental disorder with profoundly
debilitating symptoms with no FDA-approved cure or therapeutic. Brain-derived neurotrophic …

A PSD-95 peptidomimetic mitigates neurological deficits in a mouse model of Angelman syndrome

KA Lau, X Yang, MS Rioult-Pedotti, S Tang… - Progress in …, 2023 - Elsevier
Angelman Syndrome (AS) is a severe cognitive disorder caused by loss of neuronal
expression of the E3 ubiquitin ligase UBE3A. In an AS mouse model, we previously reported …

Impairment of TrkB-PSD-95 signaling in Angelman syndrome

C Cao, MS Rioult-Pedotti, P Migani, CJ Yu… - PLoS …, 2013 - journals.plos.org
Angelman syndrome (AS) is a neurodevelopment disorder characterized by severe
cognitive impairment and a high rate of autism. AS is caused by disrupted neuronal …

[HTML][HTML] Neurodevelopmental disorders and the role of PSD-95: Understanding pathways and pharmacological interventions

G Medina, AE MacKenzie - Advanced Neurology, 2024 - accscience.com
Neurodevelopmental disorders (NDDs) are often linked to disruption in brain development
and present challenges for affected individuals in achieving their cognitive, emotional, and …

Antagomir-134 as a novel therapeutic for Angelman syndrome

A Campbell - 2022 - repository.rcsi.com
Angelman syndrome is characterised by cognitive impairment with profound speech delay,
motor impairments and ataxic-like symptoms, EEG abnormalities and drug-resistant …

Behavioral deficits in an Angelman syndrome model: effects of genetic background and age

HS Huang, AJ Burns, RJ Nonneman, LK Baker… - Behavioural brain …, 2013 - Elsevier
Angelman syndrome (AS) is a severe neurodevelopmental disorder associated with
disruption of maternally inherited UBE3A (ubiquitin protein ligase E3A) expression. At the …

Angelman syndrome patient neuron screen identifies a potent and selective clinical ASO targeting UBE3A-ATS with long lasting effect in cynomolgus monkey

R Jagasia, C Bon, SV Rasmussen, S Badillo, D Tehler… - bioRxiv, 2022 - biorxiv.org
Angelman syndrome (AS) is a severe neurodevelopmental disorder caused by the loss of
neuronal E3 ligase UBE3A with no available treatment. Restoring UBE3A levels via …

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

MN Schultz, JN Crawley - Learning & Memory, 2020 - learnmem.cshlp.org
Angelman syndrome is a rare neurodevelopmental disorder caused by a mutation in the
maternal allele of the gene Ube3a. The primary symptoms of Angelman syndrome are …

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

PN Negrón-Moreno, DT Diep, CD Guoynes… - Frontiers in Behavioral …, 2022 - frontiersin.org
Angelman syndrome (AS) is a single-gene neurodevelopmental disorder associated with
cognitive and motor impairment, seizures, lack of speech, and disrupted sleep. AS is caused …

[HTML][HTML] Antisense oligonucleotide treatment rescues UBE3A expression and multiple phenotypes of an Angelman syndrome mouse model

C Milazzo, EJ Mientjes, I Wallaard, SV Rasmussen… - JCI insight, 2021 - ncbi.nlm.nih.gov
Angelman syndrome (AS) is a severe neurodevelopmental disorder for which only
symptomatic treatment with limited benefits is available. AS is caused by mutations affecting …