Danon disease

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ORPHA:34587OMIM:300257E74.0
Who is this for?
Show terms as
3Active trials19Specialists8Treatment centers

Where are you in your journey?

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

Danon disease is a rare genetic condition that mainly affects the heart, muscles, and eyes. It is sometimes called LAMP2 deficiency or glycogen storage disease type IIb. The disease is caused by changes in a gene called LAMP2, which provides instructions for making a protein that helps cells clean up and recycle waste materials. When this protein is missing or does not work properly, harmful materials build up inside cells, especially in heart and muscle cells. The most serious effect of Danon disease is on the heart. Most people develop a very thick heart muscle (called hypertrophic cardiomyopathy), which can lead to heart failure or dangerous heart rhythm problems. Muscle weakness is also common, making everyday activities harder over time. Many people also experience vision problems and some have intellectual disability or learning difficulties. Danon disease affects males and females differently because of how it is inherited. Males tend to have more severe symptoms and develop them earlier in life, often in childhood or teenage years. Females may have milder symptoms that appear later. There is currently no cure, but treatments focus on managing heart problems, supporting muscle function, and improving quality of life. Heart transplant has been used in severe cases and can significantly extend life.

Also known as:

Key symptoms:

Thickened heart muscle (hypertrophic cardiomyopathy)Heart failureIrregular or dangerously fast heartbeat (arrhythmia)Muscle weakness in the arms and legsDifficulty with physical exercise or tiring easilyVision problems, including pigment changes in the retinaIntellectual disability or learning difficulties (more common in males)Liver enlargementFainting or near-fainting episodesShortness of breathChest pain or discomfortSwelling in the legs or ankles due to heart problems

Clinical phenotype terms (6)— hover any for plain English
Cardiorespiratory arrestHP:0006543Muscle flaccidityHP:0010547
Inheritance

X-linked dominant

Carried on the X chromosome; a single copy can cause the condition

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

3 events
Dec 2023Danon Disease Natural History Study

Rocket Pharmaceuticals Inc.

TrialRECRUITING
Sep 2023A Gene Therapy Study of RP-A501 in Male Patients With Danon Disease

Rocket Pharmaceuticals Inc. — PHASE2

TrialRECRUITING
Jan 2010Precision Diagnosis and Risk Stratification of Rare Cardiomyopathies Based on Novel Cardiac Magnetic Resonance Techniques

Chinese Academy of Medical Sciences, Fuwai Hospital — NA

TrialRECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Danon disease.

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

View clinical trials →

Clinical Trials

3 recruitingView all trials with filters →
Phase 21 trial
A Gene Therapy Study of RP-A501 in Male Patients With Danon Disease
Phase 2
Actively Recruiting
PI: Joseph Rossano, MD (Children's Hospital of Philadelphia) · Sites: La Jolla, California; Boston, Massachusetts +4 more · Age: 899 yrs
N/A1 trial
Precision Diagnosis and Risk Stratification of Rare Cardiomyopathies Based on Novel Cardiac Magnetic Resonance Techniques
N/A
Actively Recruiting
PI: Minjie Lu MD, PhD · Sites: Beijing
Other1 trial
Danon Disease Natural History Study
Actively Recruiting
· Sites: Phoenix, Arizona; La Jolla, California +11 more · Age: 899 yrs

Specialists

19 foundView all specialists →
FF
Fernando de Frutos
Specialist
1 Danon disease publication
JK
Juan Pablo Kaski
Specialist
1 Danon disease publication
CH
Carolyn Y Ho
Specialist
1 Danon disease publication
KH
Kimberly N Hong
Specialist
2 Danon disease publications
MA
Michael Arad
Specialist
2 Danon disease publications
MB
Michela Brambatti
Specialist
2 Danon disease publications
BG
Barry Greenberg
Specialist
2 Danon disease publications
MT
Matthew R G Taylor
Specialist
2 Danon disease publications
PG
Pablo Garcia-Pavia
Specialist
2 Danon disease publications
EA
Eric D Adler
Specialist
2 Danon disease publications
LF
Lijun Fu
Specialist
2 Danon disease publications
EE
Emily A Eshraghian
Specialist
1 Danon disease publication
AA
Alessia Argirò
Specialist
1 Danon disease publication
QB
Quan Bui
Specialist
1 Danon disease publication
OC
Oren Caspi
Specialist
1 Danon disease publication
MP
Minjie Lu MD, PhD
Beijing
Specialist

Rare Disease Specialist

JM
Joseph Rossano, MD
PHILADELPHIA, PA
Specialist
PI on 2 active trials
PM
Priya Kishnani, M.D.
DURHAM, NC
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 Danon disease.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Danon disease

Disease timeline:

New trial: Precision Diagnosis and Risk Stratification of Rare Cardiomyopathies Based on Novel Cardiac Magnetic

Phase NA trial recruiting.

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.How severe is my heart involvement right now, and what signs should I watch for that mean it is getting worse?,Should I have an ICD implanted, and what are the risks and benefits for my situation?,At what point would you recommend a heart transplant evaluation?,Are there any clinical trials for gene therapy or other new treatments that I might qualify for?,Should my family members be tested for Danon disease, and who is most at risk?,What physical activities are safe for me, and are there any I should avoid?,What support services are available to help with school, work, or daily living?

Common questions about Danon disease

What is Danon disease?

Danon disease is a rare genetic condition that mainly affects the heart, muscles, and eyes. It is sometimes called LAMP2 deficiency or glycogen storage disease type IIb. The disease is caused by changes in a gene called LAMP2, which provides instructions for making a protein that helps cells clean up and recycle waste materials. When this protein is missing or does not work properly, harmful materials build up inside cells, especially in heart and muscle cells. The most serious effect of Danon disease is on the heart. Most people develop a very thick heart muscle (called hypertrophic cardiomy

How is Danon disease inherited?

Danon disease follows a x-linked dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Danon disease?

Yes — 3 recruiting clinical trials are currently listed for Danon disease on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Danon disease?

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