Gaucher disease type 1

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ORPHA:77259OMIM:230800E75.2
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2Active trials10Specialists8Treatment centers

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

Gaucher disease type 1 (also called non-neuronopathic Gaucher disease or adult Gaucher disease) is the most common form of Gaucher disease, a rare inherited condition where the body cannot properly break down a fatty substance called glucocerebroside. This happens because an enzyme called glucocerebrosidase does not work correctly. As a result, this fatty material builds up inside cells, especially in the liver, spleen, and bone marrow. The most noticeable effects include a very enlarged spleen and liver, low blood counts (which can cause anemia, easy bruising, and frequent infections), and bone problems such as pain, fractures, and a condition called avascular necrosis where bone tissue dies from lack of blood supply. Unlike types 2 and 3 of Gaucher disease, type 1 does not directly affect the brain or nervous system. The good news is that type 1 Gaucher disease has effective treatments available. Enzyme replacement therapy (ERT) — given as regular infusions — is the main treatment and can dramatically reduce symptoms and prevent complications. A pill-based option called substrate reduction therapy is also available for some patients. With proper treatment and monitoring, many people with Gaucher disease type 1 live full, active lives.

Also known as:

Key symptoms:

Enlarged spleen (splenomegaly), which can cause abdominal fullness or discomfortEnlarged liver (hepatomegaly)Anemia — low red blood cells causing tiredness and weaknessLow platelet count leading to easy bruising and prolonged bleedingFrequent infections due to low white blood cell countsBone pain, especially in the legs and hipsBone fractures that happen more easily than expectedAvascular necrosis — bone tissue death from poor blood supply, often in the hipFatigue and low energyDelayed growth or puberty in childrenLung problems in some patients (less common)Increased risk of a type of blood cancer called multiple myeloma or other blood disorders over time

Clinical phenotype terms (49)— hover any for plain English
HypersplenismHP:0001971Decreased beta-glucocerebrosidase levelHP:0003656
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

2 events
Jul 2024A Clinical Study Evaluating LY-M001 Injection in the Treatment of Adult Patients With Type I Gaucher Disease

Lingyi Biotech Co., Ltd. — PHASE1

TrialRECRUITING
Jan 2017Venglustat in Combination With Cerezyme in Adult Patients With Gaucher Disease Type 3 With Venglustat Monotherapy Extension

Genzyme, a Sanofi Company — PHASE2

TrialACTIVE NOT RECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Gaucher disease type 1.

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

View clinical trials →

Clinical Trials

2 recruitingView all trials with filters →
Phase 21 trial
Venglustat in Combination With Cerezyme in Adult Patients With Gaucher Disease Type 3 With Venglustat Monotherapy Extension
Phase 2
Active
PI: Clinical Sciences & Operations (Sanofi) · Sites: New Haven, Connecticut; Dallas, Texas +5 more · Age: 1899 yrs
Phase 11 trial
A Clinical Study Evaluating LY-M001 Injection in the Treatment of Adult Patients With Type I Gaucher Disease
Phase 1
Actively Recruiting
PI: Fengkui Zhang, PhD (Hematology Hospital, Chinese Academy of Medical Sc) · Sites: Guangzhou, Guangdong; Taiyuan, Shanxi +1 more · Age: 1860 yrs

Specialists

10 foundView all specialists →
NM
Noa Hurvitz, MD
Specialist
PI on 1 active trial
HM
Hamzeh Migdadi, M.D.
BROOKLYN, NY
Specialist
PI on 1 active trial
FP
Fengkui Zhang, PhD
Guangzhou, Guangdong
Specialist

Rare Disease Specialist

TM
Timothy M Cox, MD
Birmingham
Specialist

Rare Disease Specialist

PI on 1 active trial
XP
xiumin wang, PhD
Shanghai, Shanghai Municipality
Specialist

Rare Disease Specialist

PI on 1 active trial
AM
Ari Zimran, MD
Specialist
PI on 3 active trials
TP
Timothy Cox, Prof
Specialist
PI on 1 active trial
AP
Ari Zimran, Prof.
Specialist
PI on 2 active trials
EM
Edward Kaye, M.D.
CAMBRIDGE, MA
Specialist
PI on 1 active trial
HY
Hyounggyoon Yoo
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 Gaucher disease type 1.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Gaucher disease type 1

Disease timeline:

New trial: Venglustat in Combination With Cerezyme in Adult Patients With Gaucher Disease Type 3 With Venglusta

Phase PHASE2 trial recruiting. venglustat (GZ402671)

New trial: A Clinical Study Evaluating LY-M001 Injection in the Treatment of Adult Patients With Type I Gaucher

Phase PHASE1 trial recruiting. LY-M001

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 treatment option is best for me or my child, and why — enzyme replacement therapy or substrate reduction therapy?,How often will I need infusions or medication, and what does that schedule look like long-term?,What monitoring tests will I need regularly, and how often should I see a specialist?,Are my family members at risk, and should they be tested for the GBA1 gene mutation?,What bone complications should I watch for, and are there activities I should avoid?,Is there an increased risk of Parkinson's disease or cancer, and how will that be monitored?,Are there any clinical trials or new treatments I should know about?

Common questions about Gaucher disease type 1

What is Gaucher disease type 1?

Gaucher disease type 1 (also called non-neuronopathic Gaucher disease or adult Gaucher disease) is the most common form of Gaucher disease, a rare inherited condition where the body cannot properly break down a fatty substance called glucocerebroside. This happens because an enzyme called glucocerebrosidase does not work correctly. As a result, this fatty material builds up inside cells, especially in the liver, spleen, and bone marrow. The most noticeable effects include a very enlarged spleen and liver, low blood counts (which can cause anemia, easy bruising, and frequent infections), and b

How is Gaucher disease type 1 inherited?

Gaucher disease type 1 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Gaucher disease type 1?

Yes — 2 recruiting clinical trials are currently listed for Gaucher disease type 1 on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Gaucher disease type 1?

10 specialists and care centers treating Gaucher disease type 1 are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.