STT3A-CDG

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ORPHA:370921OMIM:615596E77.8
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8Treatment centers

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Overview

STT3A-CDG (also known as STT3A-Congenital Disorder of Glycosylation) is an extremely rare inherited metabolic condition that affects how the body adds sugar chains (called glycans) to proteins. This process, known as glycosylation, is essential for many proteins to work properly throughout the body. The STT3A gene provides instructions for making a key part of the protein machinery that attaches sugar chains to newly made proteins in cells. When this gene does not work correctly, many proteins throughout the body do not get their sugar chains properly attached, which can cause problems in multiple organ systems. Children with STT3A-CDG typically show signs early in life, including developmental delay, intellectual disability, low muscle tone (hypotonia), seizures, and feeding difficulties. Some patients may also have vision problems, abnormal blood clotting, and liver involvement. The severity of symptoms can vary from person to person. There is currently no cure for STT3A-CDG. Treatment focuses on managing individual symptoms and supporting development. This may include physical therapy, occupational therapy, speech therapy, anti-seizure medications, and nutritional support. Because the condition affects multiple body systems, care usually involves a team of different specialists working together. Research into congenital disorders of glycosylation is ongoing, and new therapies are being explored.

Also known as:

Key symptoms:

Developmental delayIntellectual disabilityLow muscle tone (floppy baby)SeizuresFeeding difficultiesFailure to thrive or poor growthVision problemsAbnormal blood clottingLiver problemsDelayed speechDifficulty with coordination and balanceFacial features that may appear slightly differentRecurrent infections

Clinical phenotype terms (13)— hover any for plain English
Abnormal glycosylationHP:0012345Impaired smooth pursuitHP:0007772
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 STT3A-CDG.

View clinical trials →

No actively recruiting trials found for STT3A-CDG at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the STT3A-CDG community →

No specialists are currently listed for STT3A-CDG.

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 STT3A-CDG.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about STT3A-CDG

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 specific symptoms should I watch for that might need urgent medical attention?,How often should my child have blood tests to check liver function and clotting?,What therapies (physical, occupational, speech) do you recommend and how often?,Are there any clinical trials or new treatments being studied for STT3A-CDG?,Should other family members be tested to see if they are carriers?,What is the best way to manage my child's seizures, and what should I do if a seizure lasts too long?,Can you connect us with other families affected by CDG or a CDG support organization?

Common questions about STT3A-CDG

What is STT3A-CDG?

STT3A-CDG (also known as STT3A-Congenital Disorder of Glycosylation) is an extremely rare inherited metabolic condition that affects how the body adds sugar chains (called glycans) to proteins. This process, known as glycosylation, is essential for many proteins to work properly throughout the body. The STT3A gene provides instructions for making a key part of the protein machinery that attaches sugar chains to newly made proteins in cells. When this gene does not work correctly, many proteins throughout the body do not get their sugar chains properly attached, which can cause problems in mult

How is STT3A-CDG inherited?

STT3A-CDG follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does STT3A-CDG typically begin?

Typical onset of STT3A-CDG is infantile. Age of onset can vary across affected individuals.