MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome

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Overview

MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome is a rare genetic condition that affects the muscles and joints. It is caused by changes (mutations) in both copies of the MYBPC1 gene, which provides instructions for making a protein called slow skeletal myosin-binding protein C. This protein plays an important role in how muscle fibers are organized and how muscles contract. When this protein does not work properly, muscles develop abnormally, leading to joint problems that begin before birth. The most noticeable feature of this condition is arthrogryposis multiplex congenita (AMC) — a term that means multiple joints are stuck in fixed, bent or straightened positions at birth. This happens because reduced fetal movement in the womb allows extra tissue to form around the joints. Affected individuals may have stiff joints in the arms, legs, hands, or feet, along with muscle weakness and reduced muscle mass. Some people also experience tremors (shaking) or other muscle-related symptoms. Importantly, this form of arthrogryposis is described as non-lethal, meaning most affected individuals survive into adulthood. There is currently no cure for this condition. Treatment focuses on improving joint movement, strength, and independence. This typically involves physical therapy, occupational therapy, and sometimes surgery to release tight joints or correct deformities. With early and consistent care, many people with this condition can improve their function and quality of life significantly.

Also known as:

Key symptoms:

Multiple joints fixed in bent or straightened positions at birth (arthrogryposis)Muscle weakness, especially in the limbsReduced muscle mass (thin or underdeveloped muscles)Stiff or limited joint movementClubfoot (feet turned inward or downward)Curved spine (scoliosis) in some individualsTremors or involuntary shaking of the musclesDifficulty with fine motor tasks like gripping or writingDelayed motor milestones such as sitting or walkingJoint contractures that may affect the hips, knees, elbows, or wrists

Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome.

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No actively recruiting trials found for MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome at this time.

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No specialists are currently listed for MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome.

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 MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which joints are affected in my child's case, and how severe are the contractures?,What type of genetic testing should we do to confirm the diagnosis, and should other family members be tested?,What therapies should we start right away, and how often should they happen?,Is surgery recommended, and if so, when is the best time to consider it?,What should we watch for as our child grows — are there complications that can develop over time?,Are there any clinical trials or research studies we could participate in?,What support services or patient organizations can help our family?

Common questions about MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome

What is MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome?

MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome is a rare genetic condition that affects the muscles and joints. It is caused by changes (mutations) in both copies of the MYBPC1 gene, which provides instructions for making a protein called slow skeletal myosin-binding protein C. This protein plays an important role in how muscle fibers are organized and how muscles contract. When this protein does not work properly, muscles develop abnormally, leading to joint problems that begin before birth. The most noticeable feature of this condition is arthrogry

How is MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome inherited?

MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome typically begin?

Typical onset of MYBPC1-related autosomal recessive non-lethal arthrogryposis multiplex congenita syndrome is neonatal. Age of onset can vary across affected individuals.