RASopathy

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ORPHA:536391
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6Active trials8Specialists8Treatment centers

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

RASopathies are a group of related genetic conditions caused by changes (mutations) in genes that control a key signaling pathway in the body called the RAS/MAPK pathway. This pathway acts like a communication system inside cells, helping them grow, divide, and develop normally. When genes in this pathway are altered, cells can receive too many 'grow and divide' signals, which can affect many parts of the body at once. Because of this, RASopathies are considered multi-system conditions — meaning they can impact the heart, brain, skin, muscles, bones, and more. The most well-known RASopathies include Noonan syndrome, Costello syndrome, cardio-facio-cutaneous (CFC) syndrome, Legius syndrome, and LEOPARD syndrome (also called Noonan syndrome with multiple lentigines). Each of these has its own specific features, but they share many overlapping symptoms such as distinctive facial features, heart defects, short stature, and learning differences. Some people with RASopathies also have an increased risk of certain cancers. Treatment for RASopathies is mainly focused on managing individual symptoms. There is no single cure, but many people benefit from heart surgery or medication, growth hormone therapy, educational support, and therapies for developmental delays. Research into targeted treatments that directly address the overactive RAS/MAPK pathway is ongoing, and some promising therapies are being studied in clinical trials.

Key symptoms:

Distinctive facial features such as wide-set eyes, low-set ears, and a broad foreheadHeart defects present at birth (congenital heart disease)Short stature or slow growthLearning difficulties or intellectual disability (ranges from mild to moderate)Delayed development of motor skills such as sitting, walking, or talkingSkin changes such as dark spots (lentigines), café-au-lait spots, or rough, bumpy skinLoose or flexible jointsLow muscle tone (hypotonia)Feeding difficulties in infancyIncreased risk of certain tumors or blood cancersVision or hearing problemsBleeding or clotting problemsWebbed or extra skin on the neck (pterygium colli)Chest wall abnormalities such as a sunken or raised breastboneBehavioral challenges or attention difficulties

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

6 events
Jun 2025Retrospective Natural History Study of RASopathy-associated Cardiomyopathy (RAS-CM)

Deutsches Herzzentrum Muenchen

TrialRECRUITING
Jul 2024Pubertal Development in Patients with RASopathies

IRCCS Azienda Ospedaliero-Universitaria di Bologna

TrialRECRUITING
Jan 2024Study of the Thyroid Function and Echostructural Morphology in Patients Affected With Rasopathies (ECORAS2023)

University of Bari Aldo Moro

TrialRECRUITING
Oct 2021Solid Tumors in RASopathies

Fondazione Policlinico Universitario Agostino Gemelli IRCCS — NA

TrialRECRUITING
Apr 2021Effect of RAS/MAPK Pathway Hyperactivation on Growth' and Bone' Profile of the RASopathies

Fondazione Policlinico Universitario Agostino Gemelli IRCCS — NA

TrialRECRUITING
Nov 2020Hematological Anomalies in Children With Rasopathy

Assistance Publique - Hôpitaux de Paris

TrialRECRUITING

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

Treatments

No FDA-approved treatments are currently listed for RASopathy.

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

View clinical trials →

Clinical Trials

6 recruitingView all trials with filters →
N/A2 trials
Solid Tumors in RASopathies
N/A
Actively Recruiting
PI: Chiara Leoni, MD, PhD (Fondazione Policlinico A. Gemelli, IRCCS) · Sites: Roma
Effect of RAS/MAPK Pathway Hyperactivation on Growth' and Bone' Profile of the RASopathies
N/A
Actively Recruiting
PI: Chiara Leoni, MD, PhD (Fondazione Policlinico A. Gemelli, IRCCS) · Sites: Roma
Other4 trials
Retrospective Natural History Study of RASopathy-associated Cardiomyopathy (RAS-CM)
Actively Recruiting
· Sites: München
Hematological Anomalies in Children With Rasopathy
Actively Recruiting
· Sites: Angers; Caen +12 more · Age: 015 yrs
Study of the Thyroid Function and Echostructural Morphology in Patients Affected With Rasopathies (ECORAS2023)
Actively Recruiting
PI: MARIA FELICIA FAIENZA, MD (University of Bari) · Sites: Bari · Age: 325 yrs
Pubertal Development in Patients with RASopathies
Actively Recruiting
PI: Federica Tamburrino, MD (IRCCS Azienda Ospedaliero-Universitaria di Bologna) · Sites: Bologna, Bologna · Age: 835 yrs

Specialists

8 foundView all specialists →
MM
MARIA FELICIA FAIENZA, MD
Specialist
PI on 1 active trial
FM
Federica Tamburrino, MD
Specialist
PI on 1 active trial
CP
Chiara Leoni, MD, PhD
Specialist
PI on 3 active trials
DM
Douglas R Stewart, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 5 active trials
KM
Kim E. Nichols, MD
Memphis, Tennessee
Specialist

Rare Disease Specialist

PI on 2 active trials
SP
Staci M Peron, Ph.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 8 active trials
AM
Andrew Dauber, MS MMSc
CINCINNATI, OH
Specialist
PI on 1 active trial

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 RASopathy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about RASopathy

Disease timeline:

New recruiting trial: Pubertal Development in Patients with RASopathies

A new clinical trial is recruiting patients for RASopathy

New recruiting trial: Retrospective Natural History Study of RASopathy-associated Cardiomyopathy (RAS-CM)

A new clinical trial is recruiting patients for RASopathy

New recruiting trial: Study of the Thyroid Function and Echostructural Morphology in Patients Affected With Rasopathies (ECORAS2023)

A new clinical trial is recruiting patients for RASopathy

New recruiting trial: Hematological Anomalies in Children With Rasopathy

A new clinical trial is recruiting patients for RASopathy

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.Which specific RASopathy does my child or I have, and which gene is affected?,What heart tests do I need, and how often should my heart be checked?,Is growth hormone therapy appropriate for my child's short stature?,What cancer risks are associated with my specific diagnosis, and what screening do you recommend?,What early intervention or therapy services should we start right away?,Are there any clinical trials for RASopathy treatments that we might be eligible for?,Should other family members be tested for the same genetic change?

Common questions about RASopathy

What is RASopathy?

RASopathies are a group of related genetic conditions caused by changes (mutations) in genes that control a key signaling pathway in the body called the RAS/MAPK pathway. This pathway acts like a communication system inside cells, helping them grow, divide, and develop normally. When genes in this pathway are altered, cells can receive too many 'grow and divide' signals, which can affect many parts of the body at once. Because of this, RASopathies are considered multi-system conditions — meaning they can impact the heart, brain, skin, muscles, bones, and more. The most well-known RASopathies

Are there clinical trials for RASopathy?

Yes — 6 recruiting clinical trials are currently listed for RASopathy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat RASopathy?

8 specialists and care centers treating RASopathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.