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Clinical Trials in the UK / NCT04411654
Active, not recruiting Phase 1/2

Phase 1/2 Clinical Trial of PR001 in Infants With Type 2 Gaucher Disease (PROVIDE)

NCT04411654 · tracked via the Priya Life Science UK tracker
Sponsor
Prevail Therapeutics
Phase
Phase 1/2
Started
2021-06-29
Last updated
2026-08-19

Condition(s) studied

Gaucher Disease, Type 2

Investigational drug(s) / intervention(s)

LY3884961MethylprednisoloneSirolimusPrednisone

LY3884961: Participants will receive a single dose of LY3884961 administered intracisternally.

Methylprednisolone: Single IV pulse administered as concomitant medication.

Sirolimus: Loading dose, followed by maintenance doses, followed by dose tapering; administered as concomitant medication.

Prednisone: Administered orally as concomitant medication, followed by dose tapering.

Study summary

J3Z-MC-OJAB is an open-label, Phase 1/2, multicenter study to evaluate the safety and efficacy of single-dose LY3884961 (formerly PR001) in infants diagnosed with Type 2 Gaucher disease (GD2). For each patient, the study will be approximately 5 years in duration. During the first 12 months after dosing, patients will be evaluated for the effects of LY3884961 on safety, tolerability, immunogenicity, biomarkers, and efficacy. Patients will be followed up for an additional 4 years to monitor safety and changes on selected biomarkers and clinical outcomes.

Eligibility

Sex
ALL
Min age
0 Months
Max age
24 Months
Healthy volunteers
No
Inclusion Criteria: * Bi-allelic GBA1 mutations consistent with a diagnosis of GD2 confirmed by the central laboratory. * Clinical diagnosis of GD2 * Parent/legal guardian is capable of providing signed informed consent; including compliance with the requirements and restrictions listed in the informed consent form (ICF) in this protocol. * Patient has a parent/legal guardian able to participate in the study as a source of information on the patient's health status and cognitive and functional abilities (including providing input into the rating scales). Exclusion Criteria: * Significant CNS disease other than GD2 that may be a cause for the patient's symptoms or interfere with study objectives. * Achieved independent gait. * Severe peripheral symptoms of GD which, in the opinion of the Investigator, would pose an unacceptable risk to the patient or interfere with the patient's ability to comply with study procedures or interfere with the conduct of the study. * Concomitant disease, condition, or treatment which, in the opinion of the Investigator, would pose an unacceptable risk to the patient or interfere with the patient's ability to comply with study procedures or interfere with the conduct of the study. * Use of any substrate reduction therapy (SRT) for GD treatment. * Use of prohibited medications, herbals, or over-the-counter agents as listed in the protocol. * Any type of prior gene or cell therapy. * Use of systemic immunosuppressant or corticosteroid therapy other than protocol-specified immunosuppression. * Participation in another investigational drug or device study within the past 3 months. * Brain MRI (magnetic resonance imaging) and MRA (magnetic resonance angiography) showing clinically significant abnormality deemed a contraindication to intracisternal injection. * Clinically significant laboratory test result abnormalities assessed at screening. * Contraindications or intolerance to radiographic visualization methods (e.g. MRI, MRA, CT), and intolerance to contrast agents used for MRI or CT scans. * Contraindications to general anesthesia or sedation. Other protocol-defined inclusion/exclusion criteria may apply.

Primary outcome measure(s)

Trial sites (6)

FacilityCityRegionStatus
UCSF Benioff Children's Hospital, 747 52nd St Oakland California
University of Minnesota Masonic Children's Hospital, 2450 Riverside Avenue Minneapolis Minnesota
NYU Medical Center, 305 Second Ave, Suite 16 New York New York
Children's Hospital of Pittsburgh, 4401 Penn Avenue Pittsburgh Pennsylvania
Lysosomal & Rare Disorders Research and Treatment Center Fairfax Virginia
Manchester Centre for Genomic Medicine, 6th Floor, St Mary's Hospital, Oxford Road Manchester United Kingdom

On this site

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More Prevail Therapeutics trials in the UK

Official registry record

This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.

View NCT04411654 on ClinicalTrials.gov ↗ ← All trials in the UK