NCT04519749

Brief Summary

This is a prospective multicenter, open-label, dose-escalation trial to assess the safety, tolerability, and pharmacodynamics of 4D-310 following a single IV administration. The study population is comprised of adult males and females with Fabry Disease.

Trial Health

75
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
18

participants targeted

Target at P25-P50 for phase_1

Timeline
50mo left

Started Sep 2020

Longer than P75 for phase_1

Geographic Reach
1 country

4 active sites

Status
active not recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress58%
Sep 2020Jun 2030

First Submitted

Initial submission to the registry

August 14, 2020

Completed
6 days until next milestone

First Posted

Study publicly available on registry

August 20, 2020

Completed
12 days until next milestone

Study Start

First participant enrolled

September 1, 2020

Completed
5.3 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

January 1, 2026

Completed
4.4 years until next milestone

Study Completion

Last participant's last visit for all outcomes

June 1, 2030

Expected
Last Updated

April 8, 2024

Status Verified

April 1, 2024

Enrollment Period

5.3 years

First QC Date

August 14, 2020

Last Update Submit

April 5, 2024

Conditions

Keywords

Lysosomal Storage DiseasesNervous System Brain DiseasesMetabolicInborn Brain DiseasesMetabolic Brain DiseasesCentral Nervous System DiseasesNervous System DiseasesCerebral Small Vessel DiseasesCerebrovascular DisordersVascular DiseasesCardiovascular DiseasesGenetic DiseasesX-LinkedInbornMetabolic DiseasesLipid Metabolism DisordersSphingolipidosesMetabolismInborn ErrorsLipidosesLipid Metabolism

Outcome Measures

Primary Outcomes (1)

  • Incidence and severity of adverse events

    Safety and tolerability of 4D-310 following a single IV dose, as assessed by incidence and severity of adverse events, serious adverse events and dose limiting toxicities, including clinically significant changes from baseline to scheduled time points in safety parameters

    1 year

Secondary Outcomes (2)

  • Change from baseline in serum AGA activity

    1 year

  • Change from baseline serum globotriaosylsphingosine (lysoGb3)

    1 year

Study Arms (4)

4D-310 Dose Level 1 - AAV Neutralizing Antibody (NAb) Group A

EXPERIMENTAL

Single IV administration of 4D-310 Dose Level 1 - AAV NAb Titer Group A patients

Biological: 4D-310

4D-310 Dose Level 1 - AAV NAb Titer Group B

EXPERIMENTAL

Single IV administration of 4D-310 Dose Level 1 - AAV NAb titer Group B patients

Biological: 4D-310

4D-310 Dose Level 2 - AAV NAb Titer Group A and/or B

EXPERIMENTAL

Single IV administration of 4D-310 at Dose Level 2 in AAV NAb titer Group A and/or B patients

Biological: 4D-310

4D-310 Dose Expansion

EXPERIMENTAL

Dose expansion cohort of single IV administration of 4D-310 at the selected dose and selected AAV Nab titer group(s) patients

Biological: 4D-310

Interventions

4D-310BIOLOGICAL

4D-310 is a novel adeno-associated virus (AAV) gene therapy comprised of two active components: the capsid (4D-C102) and the transgene cassette, which encodes a codon-optimized full length human GLA transgene driven by the CAG promoter. 4D-310 has been engineered so that it cannot replicate (replication incompetent).

4D-310 Dose Expansion4D-310 Dose Level 1 - AAV NAb Titer Group B4D-310 Dose Level 1 - AAV Neutralizing Antibody (NAb) Group A4D-310 Dose Level 2 - AAV NAb Titer Group A and/or B

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Male or female ≥ 18 years of age
  • Pathogenic GLA mutation consistent with Fabry Disease
  • Confirmed diagnosis of classic or late-onset Fabry disease
  • Individuals on ERT must be on a stable dose for at least 6 months (and a minimum of 12 months total exposure) prior to study enrollment
  • Agree to use highly effective contraception

You may not qualify if:

  • Presence of high titer neutralizing antibody to 4D-310 capsid, or presence of high antibody titer to AGA
  • eGFR \<45 mL/min/1.73 m2
  • Undergone kidney transplantation or currently on hemodialysis or peritoneal dialysis
  • HIV, active or chronic hepatitis B or C,
  • Evidence of liver disease, severe pulmonary disease or diabetes with poor glycemic control
  • History of stroke or transient ischemic attack within the last 12 months, or other significant thromboembolic disease history (e.g. pulmonary embolism)
  • Contraindication to systemic corticosteroid therapy or immunosuppressive therapy
  • Chronic steroid use, defined as ≥ 3 months of oral corticosteroid use within the last 12 months.
  • Moderately severe to severe cardiovascular disease or uncontrolled hypertension
  • Left ventricular ejection fraction of \<45% on echocardiogram (ECHO)
  • Currently receiving investigational drug, device or therapy or having ever received gene therapy
  • History of infusion related response to ERT or any adverse reaction leading to ERT discontinuation
  • History of cancer within 2 years (exceptions include non-melanoma skin cancer, localized prostate cancer treated with curative intent)
  • Pregnant or breast-feeding

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (4)

University of California at San Diego

La Jolla, California, 92037, United States

Location

Emory University

Atlanta, Georgia, 30322, United States

Location

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, 15224, United States

Location

Lysosomal & Rare Disorders Research & Treatment Center, Inc

Fairfax, Virginia, 22030, United States

Location

MeSH Terms

Conditions

Fabry DiseaseLysosomal Storage DiseasesBrain Diseases, MetabolicCentral Nervous System DiseasesNervous System DiseasesCerebral Small Vessel DiseasesCerebrovascular DisordersVascular DiseasesCardiovascular DiseasesGenetic Diseases, InbornMetabolic DiseasesLipid Metabolism DisordersSphingolipidosesLipidoses

Condition Hierarchy (Ancestors)

Lysosomal Storage Diseases, Nervous SystemBrain Diseases, Metabolic, InbornBrain DiseasesGenetic Diseases, X-LinkedCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolism, Inborn ErrorsLipid Metabolism, Inborn ErrorsNutritional and Metabolic Diseases

Study Officials

  • Alan H Cohen, MD

    4D Molecular Therapeutics

    STUDY DIRECTOR

Study Design

Study Type
interventional
Phase
phase 1
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
SEQUENTIAL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

August 14, 2020

First Posted

August 20, 2020

Study Start

September 1, 2020

Primary Completion

January 1, 2026

Study Completion (Estimated)

June 1, 2030

Last Updated

April 8, 2024

Record last verified: 2024-04

Data Sharing

IPD Sharing
Will not share

Locations