Angelman syndrome due to a point mutation

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ORPHA:411511OMIM:105830Q93.5
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11Active trials8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Angelman syndrome due to a point mutation is a specific genetic form of Angelman syndrome, a neurological condition that affects the nervous system and causes significant developmental challenges. In this form, the disease is caused by a small change (point mutation) in the UBE3A gene on chromosome 15. This gene normally helps the brain develop and function properly, but when it is altered by a mutation, the brain cannot work as expected. People with Angelman syndrome typically show severe intellectual disability, little or no speech, a happy and excitable demeanor with frequent smiling and laughter, balance and movement problems, and seizures. Symptoms usually become noticeable between 6 and 12 months of age when developmental delays appear. Children may have trouble sitting, crawling, or walking, and speech development is severely limited or absent. There is currently no cure for Angelman syndrome. Treatment focuses on managing symptoms such as seizures with anti-epileptic medications, physical therapy to improve movement, speech therapy using alternative communication methods, and behavioral support. Ongoing research into gene therapy and other approaches offers hope for future disease-modifying treatments. The point mutation form of Angelman syndrome is particularly important to identify because it carries a higher recurrence risk in families compared to the more common deletion form.

Key symptoms:

Severe intellectual disabilityLittle or no speechFrequent smiling and laughterEasily excitable personalityBalance and walking difficulties (ataxia)Seizures or epilepsySmall head size (microcephaly)Sleep problemsJerky or stiff movementsHand flappingFascination with waterFeeding difficulties in infancyTongue thrustingWide-based walking gaitHyperactivity and short attention span

Clinical phenotype terms (34)— hover any for plain English
Cessation of head growthHP:0004485Happy demeanorHP:0040082Mild microcephalyHP:0040196Inappropriate laughterHP:0000748
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Mar 2026A Natural History Study of Angelman Syndrome

Massachusetts General Hospital

TrialRECRUITING
Oct 2025A Phase 1/2 Study of the Safety and Efficacy of MVX-220 in Angelman Syndrome

MavriX Bio, LLC — PHASE1, PHASE2

TrialRECRUITING
Oct 2025A Safety and Efficacy Study of GTX-102 in Subjects With Deletion- or Nondeletion-type Angelman Syndrome (AS)

Ultragenyx Pharmaceutical Inc — PHASE2

TrialRECRUITING
Feb 2025Use of Eye Tracking to Study Social Perception Abnormalities in Children With Angelman Syndrome

Assistance Publique - Hôpitaux de Paris

TrialRECRUITING
Dec 2024Phase 3 Efficacy and Safety Study of GTX-102 in Pediatric Subjects With Angelman Syndrome (AS)

Ultragenyx Pharmaceutical Inc — PHASE3

TrialACTIVE NOT RECRUITING
Jul 2024Long-term Extension of GTX-102 in Angelman Syndrome

Ultragenyx Pharmaceutical Inc — PHASE3

TrialENROLLING BY INVITATION
Dec 2023Angelman Natural History Study - FAST Spain

Puerta de Hierro University Hospital

TrialRECRUITING
Mar 2022Study of the Prevalence of Autistic Traits in Angelman Syndrome

IRCCS Eugenio Medea

TrialRECRUITING
Dec 2021HALOS: A Safety, Tolerability, Pharmacokinetics and Pharmacodynamics Study of Multiple Ascending Doses of ION582 in Participants With Angelman Syndrome

Ionis Pharmaceuticals, Inc. — PHASE1, PHASE2

TrialRECRUITING
Oct 2021Natural History Study for Patients With Angelman Syndrome

Centre Hospitalier Universitaire de Liege

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Angelman syndrome due to a point mutation.

11 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

11 recruitingView all trials with filters →
Phase 32 trials
Phase 3 Efficacy and Safety Study of GTX-102 in Pediatric Subjects With Angelman Syndrome (AS)
Phase 3
Active
PI: Medical Director (Ultragenyx Pharmaceuticals Inc.) · Sites: Los Angeles, California; San Diego, California +26 more · Age: 417 yrs
Long-term Extension of GTX-102 in Angelman Syndrome
Phase 3
Enrolling by Invitation
PI: Medical Director (Ultragenyx Pharmaceuticals Inc.) · Sites: Los Angeles, California; San Diego, California +21 more
Phase 21 trial
A Safety and Efficacy Study of GTX-102 in Subjects With Deletion- or Nondeletion-type Angelman Syndrome (AS)
Phase 2
Actively Recruiting
PI: Medical Director (Ultragenyx Pharmaceutical Inc) · Sites: Los Angeles, California; Chicago, Illinois +20 more · Age: 164 yrs
Other6 trials
A Natural History Study of Angelman Syndrome
Actively Recruiting
PI: Christopher J Keary, MD (Massachusetts General Hospital) · Sites: Lexington, Massachusetts · Age: 199 yrs
Study of the Prevalence of Autistic Traits in Angelman Syndrome
Actively Recruiting
PI: Martina Baggio (Scientific Institute IRCCS E. Medea, Epilepsy Unit) · Sites: Conegliano, Treviso · Age: 414 yrs
Use of Eye Tracking to Study Social Perception Abnormalities in Children With Angelman Syndrome
Actively Recruiting
PI: Nathalie MD, PhD Boddaert (Assistance Publique - Hôpitaux de Paris) · Sites: Paris · Age: 317 yrs
Angelman Natural History Study - FAST Spain
Actively Recruiting
PI: BELEN RUIZ ANTORAN, MD, Ph (Clinical Pharmacology Deparment, Hospital Universi) · Sites: Barcelona; Madrid · Age: 099 yrs
Natural History Study for Patients With Angelman Syndrome
Actively Recruiting
PI: Laura Vanden Brande (CHR Citadelle) · Sites: Liège, Liège
The Global Angelman Syndrome Registry
Actively Recruiting
PI: Helen (Honey) Heussler, MBBS, FRACP DM (The University of Queensland) · Sites: Brisbane, Queensland

No specialists are currently listed for Angelman syndrome due to a point mutation.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Angelman syndrome due to a point mutation.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Is my child's specific UBE3A mutation inherited, and what does this mean for future pregnancies?,What is the best seizure management plan, and what medications should be avoided?,What therapies should we start right away to support development?,Are there any clinical trials or emerging treatments my child might be eligible for?,What communication tools or devices would you recommend for my child?,How should we manage sleep problems, and is melatonin appropriate?,What should our long-term care plan look like as my child grows into adulthood?

Common questions about Angelman syndrome due to a point mutation

What is Angelman syndrome due to a point mutation?

Angelman syndrome due to a point mutation is a specific genetic form of Angelman syndrome, a neurological condition that affects the nervous system and causes significant developmental challenges. In this form, the disease is caused by a small change (point mutation) in the UBE3A gene on chromosome 15. This gene normally helps the brain develop and function properly, but when it is altered by a mutation, the brain cannot work as expected. People with Angelman syndrome typically show severe intellectual disability, little or no speech, a happy and excitable demeanor with frequent smiling and l

How is Angelman syndrome due to a point mutation inherited?

Angelman syndrome due to a point mutation follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Angelman syndrome due to a point mutation typically begin?

Typical onset of Angelman syndrome due to a point mutation is infantile. Age of onset can vary across affected individuals.

Are there clinical trials for Angelman syndrome due to a point mutation?

Yes — 11 recruiting clinical trials are currently listed for Angelman syndrome due to a point mutation on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.