SIN3-related intellectual disability syndrome due to a point mutation

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ORPHA:500166OMIM:613406Q87.8
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Overview

SIN3-related intellectual disability syndrome due to a point mutation (also sometimes called SIN3A syndrome or Witteveen-Kolk syndrome when caused by deletions, though the point mutation form is a closely related but distinct condition) is a rare genetic disorder caused by a change in the SIN3A gene. This gene plays an important role in controlling how other genes are switched on and off in the body, especially in the developing brain. When the SIN3A gene has a point mutation — meaning a single 'letter' in the DNA code is changed — it does not work properly, and this affects brain development and function. The most noticeable effect of this condition is intellectual disability, which can range from mild to moderate. Many people with this syndrome also have delayed speech and language development, behavioral challenges, and features that affect their physical appearance. Some individuals may have autism spectrum disorder or attention difficulties. The condition is present from birth, though it is often recognized during early childhood when developmental delays become more apparent. There is currently no cure for SIN3-related intellectual disability syndrome. Treatment focuses on supporting development and managing symptoms. This includes speech therapy, occupational therapy, physical therapy, special education programs, and behavioral support. With the right support, many individuals can make meaningful progress and improve their quality of life.

Key symptoms:

Intellectual disability (difficulty with learning and thinking)Delayed speech and language developmentDelayed walking or other motor milestonesBehavioral challenges, including hyperactivity or attention difficultiesFeatures of autism spectrum disorder, such as difficulty with social interactionMild differences in facial appearance (such as widely spaced eyes or a broad forehead)Short statureFeeding difficulties in infancyAnxiety or emotional regulation difficultiesRepetitive behaviors

Clinical phenotype terms (20)— hover any for plain English
Mild malformation of cortical developmentHP:0032059Decreased head circumferenceHP:0040195Fragile nailsHP:0001808Dysplastic corpus callosumHP:0006989
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 ↗

Treatments

No FDA-approved treatments are currently listed for SIN3-related intellectual disability syndrome due to a point mutation.

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No actively recruiting trials found for SIN3-related intellectual disability syndrome due to a point mutation at this time.

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No specialists are currently listed for SIN3-related intellectual disability 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 SIN3-related intellectual disability syndrome due to a point mutation.

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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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Family & Caregiver Grants

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.What therapies should my child start right away, and how often should they happen?,Should other family members be tested for this gene change?,What signs should I watch for that might mean my child's condition is changing or getting worse?,Are there any clinical trials or research studies we could participate in?,How can I get an individualized education plan (IEP) set up for my child at school?,What specialists should be part of my child's care team, and how often should we see them?,Are there any support groups or patient organizations for families affected by SIN3A-related conditions?

Common questions about SIN3-related intellectual disability syndrome due to a point mutation

What is SIN3-related intellectual disability syndrome due to a point mutation?

SIN3-related intellectual disability syndrome due to a point mutation (also sometimes called SIN3A syndrome or Witteveen-Kolk syndrome when caused by deletions, though the point mutation form is a closely related but distinct condition) is a rare genetic disorder caused by a change in the SIN3A gene. This gene plays an important role in controlling how other genes are switched on and off in the body, especially in the developing brain. When the SIN3A gene has a point mutation — meaning a single 'letter' in the DNA code is changed — it does not work properly, and this affects brain development

How is SIN3-related intellectual disability syndrome due to a point mutation inherited?

SIN3-related intellectual disability 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 SIN3-related intellectual disability syndrome due to a point mutation typically begin?

Typical onset of SIN3-related intellectual disability syndrome due to a point mutation is infantile. Age of onset can vary across affected individuals.