Gaucher disease type 3

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ORPHA:77261OMIM:231000E75.2
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6Active trials14Specialists8Treatment 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 3 (also called chronic neuronopathic Gaucher disease or type 3 Gaucher disease) is 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, bone marrow, and brain. Gaucher disease has three main types, and type 3 is unique because it affects both the body and the nervous system, though more slowly than type 2. People with Gaucher disease type 3 often have an enlarged liver and spleen, bone pain, anemia, and low platelet counts that can cause easy bruising or bleeding. The nervous system problems — which set type 3 apart — can include abnormal eye movements, seizures, difficulty with coordination, and in some cases, gradual decline in thinking and learning abilities. Symptoms usually begin in childhood but can vary widely from person to person. Treatment has improved greatly over the past few decades. Enzyme replacement therapy (ERT) — using medicines like imiglucerase, velaglucerase alfa, or taliglucerase alfa — can effectively treat the body-related symptoms. Substrate reduction therapy (SRT) with eliglustat or miglustat is another option for some patients. However, these treatments do not fully reach the brain, so the neurological symptoms remain harder to treat. Research into better therapies for the brain-related aspects of this disease is ongoing.

Also known as:

Key symptoms:

Enlarged spleen (splenomegaly) causing abdominal fullness or discomfortEnlarged liver (hepatomegaly)Anemia causing tiredness and pale skinLow platelet counts leading to easy bruising or bleedingBone pain and increased risk of fracturesAbnormal horizontal eye movements (oculomotor apraxia)SeizuresProblems with coordination and balance (ataxia)Muscle stiffness or spasticitySlowed thinking or learning difficultiesDelayed growth and development in childrenDifficulty swallowing in some casesFatigue and low energy

Clinical phenotype terms (40)— hover any for plain English
Increased susceptibility to fracturesHP:0002659
Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

6 events
Mar 2025High-Dose Ambroxol in Pediatric Type III Gaucher Disease (GD3)

Agyany Pharma LTD — NA

TrialACTIVE NOT RECRUITING
Apr 2024Effect of Enzyme Replacement Therapy on Cardiac Function in Children With Gaucher Disease Type 3

Noha Osman — NA

TrialENROLLING BY INVITATION
Nov 2023A Study of the Safety and Preliminary Efficacy of LY-M001 Injection in the Treatment of Adult Patients with Gaucher Disease Type I

He Huang — NA

TrialRECRUITING
Jan 2023MAGNETIC RESONANCE SPECTROSCOPY BIOMARKERS IN TYPE 3 GAUCHER DISEASE (GD3)

University of Minnesota — NA

TrialRECRUITING
Apr 2022Study to Evaluate the Efficacy and Safety of Venglustat in Adult and Pediatric Patients With Gaucher Disease Type 3

Sanofi — PHASE3

TrialACTIVE NOT RECRUITING
Jan 2014Gaucherite - A Study to Stratify Gaucher Disease

Cambridge University Hospitals NHS Foundation Trust — NA

TrialRECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Gaucher disease type 3.

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

View clinical trials →

Clinical Trials

6 recruitingView all trials with filters →
Phase 31 trial
Study to Evaluate the Efficacy and Safety of Venglustat in Adult and Pediatric Patients With Gaucher Disease Type 3
Phase 3
Active
PI: Clinical Sciences & Operations (Sanofi) · Sites: New Haven, Connecticut; Iowa City, Iowa +18 more · Age: 1299 yrs
N/A5 trials
Effect of Enzyme Replacement Therapy on Cardiac Function in Children With Gaucher Disease Type 3
N/A
Enrolling by Invitation
· Sites: Asyut
Gaucherite - A Study to Stratify Gaucher Disease
N/A
Actively Recruiting
PI: Timothy M Cox, MD (University of Cambridge) · Sites: Birmingham; Birmingham +6 more
A Study of the Safety and Preliminary Efficacy of LY-M001 Injection in the Treatment of Adult Patients with Gaucher Disease Type I
N/A
Actively Recruiting
PI: He Huang, PhD · Sites: Hanzhou, Zhejiang · Age: 1860 yrs
MAGNETIC RESONANCE SPECTROSCOPY BIOMARKERS IN TYPE 3 GAUCHER DISEASE (GD3)
N/A
Actively Recruiting
PI: Reena Kartha, PhD · Sites: Minneapolis, Minnesota · Age: 1880 yrs
High-Dose Ambroxol in Pediatric Type III Gaucher Disease (GD3)
N/A
Active
PI: Huma Arshad Cheema, Prof. (The Children's Hospital, Lahore, Pakistan) · Sites: Lahore · Age: 318 yrs

Specialists

14 foundView all specialists →
KM
Karin Ludwig, M.D.
Specialist
PI on 1 active trial
AZ
Ari Zimran
Specialist
PI on 2 active trials
TM
Timothy M Cox, MD
Birmingham
Specialist

Rare Disease Specialist

PI on 1 active trial
TM
Tippi C MacKenzie, MD
San Francisco, California
Specialist

Rare Disease Specialist

PI on 2 active trials
BM
Brian Kushner, MD
NEW YORK, NY
Specialist
PI on 8 active trials
TM
Tippi MacKenzie, MD
San Francisco, California
Specialist

Rare Disease Specialist

PI on 2 active trials
HP
Huma Arshad Cheema, Prof.
Lahore, NY
Specialist

Rare Disease Specialist

PI on 1 active trial
NP
Ni-Chung Lee, M.D., Ph.D
Specialist
PI on 2 active trials
AP
Ari Zimran, Prof.
Specialist
PI on 2 active trials
RP
Reena Kartha, PhD
Minneapolis, Minnesota
Specialist

Rare Disease Specialist

PI on 2 active trials
PM
Paul Chapman, MD
Specialist
PI on 4 active trials
WM
William Tap, M.D.
NEW YORK, NY
Specialist
PI on 1 active trial

Treatment Centers

8 centers
⚗️ Trial Site

University of Minnesota

📍 Minneapolis, Minnesota

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🔬 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

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

Travel Grants

No travel grants are currently matched to Gaucher disease type 3.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Gaucher disease type 3

Disease timeline:

New trial: MAGNETIC RESONANCE SPECTROSCOPY BIOMARKERS IN TYPE 3 GAUCHER DISEASE (GD3)

Phase NA trial recruiting. No intervention

New trial: Gaucherite - A Study to Stratify Gaucher Disease

Phase NA trial recruiting. Stratified response to Enzyme Therapy

New trial: Effect of Enzyme Replacement Therapy on Cardiac Function in Children With Gaucher Disease Type 3

Phase NA trial recruiting. echo

New trial: High-Dose Ambroxol in Pediatric Type III Gaucher Disease (GD3)

Phase NA trial recruiting. Ambroxol

New trial: Study to Evaluate the Efficacy and Safety of Venglustat in Adult and Pediatric Patients With Gaucher

Phase PHASE3 trial recruiting. Venglustat

New trial: A Study of the Safety and Preliminary Efficacy of LY-M001 Injection in the Treatment of Adult Patien

Phase NA trial recruiting. LY-M001 Injection

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 GBA1 mutations does my child or I have, and what does that mean for how the disease will progress?,Which enzyme replacement therapy is recommended for us, and how often will infusions be needed?,How will you monitor the neurological symptoms over time, and what signs should prompt us to contact you urgently?,Are there any clinical trials for treatments that target the brain symptoms of Gaucher type 3 that we could join?,What support services — such as physiotherapy, educational support, or psychological counseling — should we be accessing?,Should other family members be tested for the GBA1 gene mutation?,What is the long-term plan for managing this condition as my child grows into adulthood?

Common questions about Gaucher disease type 3

What is Gaucher disease type 3?

Gaucher disease type 3 (also called chronic neuronopathic Gaucher disease or type 3 Gaucher disease) is 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, bone marrow, and brain. Gaucher disease has three main types, and type 3 is unique because it affects both the body and the nervous system, though more slowly than type 2. People with Gaucher disease type 3 ofte

How is Gaucher disease type 3 inherited?

Gaucher disease type 3 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Gaucher disease type 3 typically begin?

Typical onset of Gaucher disease type 3 is childhood. Age of onset can vary across affected individuals.

Are there clinical trials for Gaucher disease type 3?

Yes — 6 recruiting clinical trials are currently listed for Gaucher disease type 3 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 3?

14 specialists and care centers treating Gaucher disease type 3 are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.