NCT04093349

Brief Summary

The purpose of this study is to evaluate the safety, tolerability, and efficacy of a single intravenous infusion of SPK-3006 in adults with clinically moderate, late-onset Pompe disease receiving enzyme replacement therapy (ERT). Participants will be treated in sequential, dose-level cohorts.

Trial Health

82
On Track

Trial Health Score

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

Enrollment
4

participants targeted

Target at below P25 for phase_1

Timeline
72mo left

Started Oct 2020

Longer than P75 for phase_1

Geographic Reach
8 countries

29 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 Progress49%
Oct 2020Apr 2032

First Submitted

Initial submission to the registry

September 16, 2019

Completed
2 days until next milestone

First Posted

Study publicly available on registry

September 18, 2019

Completed
1 year until next milestone

Study Start

First participant enrolled

October 1, 2020

Completed
11.5 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

April 1, 2032

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

April 1, 2032

Last Updated

November 27, 2024

Status Verified

November 1, 2024

Enrollment Period

11.5 years

First QC Date

September 16, 2019

Last Update Submit

November 25, 2024

Conditions

Outcome Measures

Primary Outcomes (3)

  • Number of adverse and serious adverse events (AEs/SAEs), including clinically significant abnormal laboratory values.

    Adverse events.

    Up to 5 years

  • Occurrence of immune response against AAV capsid

    Up to 5 years

  • Occurrence of immune response against GAA transgene

    Up to 5 years

Study Arms (1)

SPK-3006

EXPERIMENTAL

All participants who meet the eligibility criteria will receive a single intravenous (i.v.) administration of SPK-3006.

Genetic: SPK-3006

Interventions

SPK-3006GENETIC

adeno-associated viral (AAV) vector

SPK-3006

Eligibility Criteria

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

You may qualify if:

  • Provide written informed consent;
  • Males and Females ≥18 years of age with late-onset Pompe disease;
  • Received ERT for at least the previous 24 months
  • Have clinically moderate, late-onset Pompe disease characteristics;
  • Agree to use reliable contraception.

You may not qualify if:

  • Active hepatitis B and/or C;
  • Significant underlying liver disease;
  • Human immunodeficiency virus (HIV) infection;
  • Prior hypersensitivity to rhGAA;
  • Pre-existing anti-AAV neutralizing antibody titers;
  • High titer antibody responses to rhGAA;
  • Requires any invasive ventilation or requires noninvasive ventilation while awake and upright;
  • Received any prior vector or gene transfer agent;
  • Active malignancy (except non-melanoma skin cancer);
  • History of liver cancer;
  • Pregnant or nursing women;
  • Any evidence of active infection at the time of SPK-3006 infusion.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (29)

Barrow Neurological Institute

Phoenix, Arizona, 85013, United States

Location

University of California Irvine Health

Orange, California, 92868, United States

Location

Emory University School of Medicine

Atlanta, Georgia, 30329, United States

Location

University of Kansas Medical Center Research Institute

Kansas City, Kansas, 66160, United States

Location

University of Minnesota

Minneapolis, Minnesota, 55455, United States

Location

Oregon Health & Science University

Portland, Oregon, 97239, United States

Location

University of Pennsylvania

Philadelphia, Pennsylvania, 19104, United States

Location

University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, 15213, United States

Location

University of Utah

Salt Lake City, Utah, 84108, United States

Location

Lysosomal and Rare Disorders Research & Treatment Center

Fairfax, Virginia, 22030, United States

Location

Vancouver General Hospital

Vancouver, British Columbia, V5Z 1M9, Canada

Location

The Ottawa Hospital

Ottawa, Ontario, K1Y 4E9, Canada

Location

Montreal Neurological Hospital

Montreal, Quebec, H3A 2B4, Canada

Location

Rigshospitalet

Copenhagen, 84 2100, Denmark

Location

Centre Hospitalier Universitaire d'Angers

Angers, 49933, France

Location

CHU Paris IdF Ouest - Hôpital Raymond Poincaré

Garches, 92380, France

Location

Centre Hospitalier Régional Universitaire de Lille

Lille, 59037, France

Location

Assistance Publique Hôpitaux de Marseille

Marseille, 13 13385, France

Location

Nice University Hospital

Nice, 6000, France

Location

Friedrich-Baur-Institut Neurologische Klinik Ludwig-Maximilians-Universität München

München, D08033, Germany

Location

Universita Degli Studi Di Messina - Dipartimento di Medicina Clinica e Sperimentale

Messina, 98122, Italy

Location

Universita Degli Studi Di Milano, Laboratorio di Biochimica e Genetica della Malattie Neuromuscolari

Milan, 35 20122, Italy

Location

Malattie Metaboliche Universita Degli Studi Di Napoli Federico II

Naples, 80138, Italy

Location

UO Neurologia Azienda Ospedaliera Universitaria Pisana

Pisa, 56126, Italy

Location

Azienda Ospedaliera Universitaria Città della Salute e della Scienza di Torino - Neurology, Osp. Molinette

Torino, 10126, Italy

Location

Erasmus Medical Center

Rotterdam, 3015 CE, Netherlands

Location

New Queen Elizabeth Hospital Birmingham

Birmingham, GBR, B15 2WB, United Kingdom

Location

The Royal Free London NHS Foundation Trust

London, GBR, NW3 2QG, United Kingdom

Location

Salford Royal MHS Foundation Trust

Salford, M6 8HD, United Kingdom

Location

MeSH Terms

Conditions

Glycogen Storage Disease Type IILysosomal Storage Diseases

Condition Hierarchy (Ancestors)

Lysosomal Storage Diseases, Nervous SystemBrain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesMetabolism, Inborn ErrorsGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesGlycogen Storage DiseaseCarbohydrate Metabolism, Inborn ErrorsMetabolic DiseasesNutritional and Metabolic Diseases

Study Officials

  • Tahseen Mozaffar, MD

    University of California Irvine Health

    PRINCIPAL INVESTIGATOR

Study Design

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

Study Record Dates

First Submitted

September 16, 2019

First Posted

September 18, 2019

Study Start

October 1, 2020

Primary Completion (Estimated)

April 1, 2032

Study Completion (Estimated)

April 1, 2032

Last Updated

November 27, 2024

Record last verified: 2024-11

Data Sharing

IPD Sharing
Will not share

Locations