147 Participants Needed

Oral Nizubaglustat for Niemann-Pick Disease

Recruiting at 35 trial locations
Cf
PA
Overseen ByPatient Advocacy Representative
Age: Any Age
Sex: Any
Trial Phase: Phase 3
Sponsor: Azafaros A.G.
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)
Pivotal Trial (Near Approval)This treatment is in the last trial phase before FDA approval
Prior Safety DataThis treatment has passed at least one previous human trial

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial tests a new oral treatment, nizubaglustat (AZ-3102), for two rare conditions: Niemann-Pick type C disease and GM1 or GM2 gangliosidosis. The goal is to determine if this treatment is safe and effective for individuals with the late-infantile or juvenile forms of these diseases. Participants will be randomly assigned to receive either the treatment or a placebo (a pill with no active drug) for comparison. The trial seeks individuals aged 4 and older who have been diagnosed with one of these conditions. As a Phase 3 trial, this study represents the final step before FDA approval, offering participants a chance to contribute to a potentially groundbreaking treatment.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications. Please refer to the detailed subprotocols or consult with the trial coordinators for more information.

Is there any evidence suggesting that this trial's treatments are likely to be safe?

Research has shown that nizubaglustat is safe and well-tolerated. One study found that people with Niemann-Pick disease type C and GM2 gangliosidosis experienced no major side effects from the treatment. This suggests nizubaglustat is generally manageable for patients.

Although minor side effects might occur, the data supports further development of this treatment. Nizubaglustat has demonstrated sufficient safety evidence to continue in clinical research. Prospective trial participants may find this information reassuring. However, discussing any concerns with a doctor is always important.12345

Why do researchers think this study treatment might be promising for Niemann-Pick disease?

Unlike the standard treatments for Niemann-Pick Disease, which often focus on managing symptoms or slowing disease progression, nizubaglustat targets the disease at a molecular level. It works by inhibiting an enzyme involved in the abnormal lipid storage that characterizes this condition. This novel mechanism of action offers the potential to address the root cause of the disease, rather than just alleviating symptoms. Researchers are excited because this could lead to more effective and long-lasting outcomes for patients.

What evidence suggests that this treatment might be an effective treatment for Niemann-Pick disease?

This trial will evaluate nizubaglustat for Niemann-Pick disease type C and GM1/GM2 gangliosidoses. Studies have shown that nizubaglustat, the treatment being tested, has promising early results for these conditions. In previous trials, patients demonstrated positive signs that the treatment might help manage these diseases. Nizubaglustat targets specific processes in the body involved in these diseases, potentially slowing their progression. Research from earlier studies indicates it is generally safe and well-tolerated by patients. While more information is needed, these initial findings offer hope that nizubaglustat could be an effective option for these rare diseases.26789

Are You a Good Fit for This Trial?

This trial is for children and young adults with late-infantile and juvenile forms of Niemann-Pick Type C disease, GM1 Gangliosidosis, or GM2 Gangliosidosis. Specific eligibility criteria are not provided but typically include a confirmed diagnosis and meeting certain health standards.

Inclusion Criteria

I am 4 years or older with late-infantile or juvenile NPC disease.
I am 4 years or older with a late-infantile/juvenile onset of GM1 or GM2 gangliosidosis.

Exclusion Criteria

Detailed exclusion criteria are presented in the GM1/GM2 gangliosidosis-specific subprotocol AZA-001-301-GMx
Detailed exclusion criteria are presented in the NPC disease-specific subprotocol AZA-001-301-NPC

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Participants receive oral nizubaglustat or placebo for 18 months in a double-blind, randomized, placebo-controlled setting

18 months

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

  • AZ-3102
Trial Overview The study tests the safety and effectiveness of an oral medication called Nizubaglustat (AZ-3102) compared to a placebo over 18 months. Participants will be randomly assigned to either the drug or placebo group in a double-blind manner, meaning neither they nor the researchers know who's receiving which treatment.
How Is the Trial Designed?
2Treatment groups
Experimental Treatment
Group I: Subprotocol NCT07082725: Intervention Group NPCExperimental Treatment2 Interventions
Group II: Subprotocol NCT07082543: Intervention Group GM1/GM2Experimental Treatment2 Interventions

Find a Clinic Near You

Who Is Running the Clinical Trial?

Azafaros A.G.

Lead Sponsor

Trials
2
Recruited
40+

Citations

Azafaros Announces Initiation of two Global Phase 3 ...Nizubaglustat has received Rare Pediatric Disease Designations (RPDD) for the treatment of GM1 and GM2 gangliosidoses and NPC, Orphan Drug ...
NCT07082543 | A Study to Evaluate the Safety and ...A Study to Evaluate the Safety and Efficacy of Oral Nizubaglustat (AZ-3102) in Late-infantile and Juvenile Forms of GM1 Gangliosidosis or GM2 Gangliosidosis.
Oral Nizubaglustat (AZ-3102) in Late-infantile and Juvenile ...A Study to Evaluate the Safety and Efficacy of Oral Nizubaglustat (AZ-3102) in Late-infantile and Juvenile Forms of GM1 Gangliosidosis or GM2 Gangliosidosis.
Study on the Effectiveness and Safety of Nizubaglustat in ...This clinical trial investigates the effectiveness and safety of nizubaglustat in treating Niemann-Pick Type C, GM1, and GM2 Gangliosidosis, ...
NCT07054515 | A Study to Evaluate the Safety and ...This is a Phase 3 randomized, double-blinded, placebo-controlled study that will evaluate the safety and efficacy of oral nizubaglustat (AZ-3102) in multiple ...
Azafaros announces positive topline Phase 2 study data ...Study designed to support safety profile showed nizubaglustat was safe and well tolerated; Results support continued development of ...
Newsletter for patients on Azafaros' current programUpdate on PRONTO and Phase 2 RAINBOW study data with nizubaglustat, for patients with Niemann-Pick disease type C (NPC). GM1 and GM2 gangliosidoses. In ...
Azafaros Announces Initiation of two Global Phase 3 studiesNizubaglustat has received Rare Pediatric Disease Designations (RPDD) for the treatment of GM1 and GM2 gangliosidoses and NPC, Orphan Drug ...
NCT05758922 | Phase 2 Study Evaluating the Safety, ...Phase 2 Study Evaluating the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Oral AZ-3102 in Patients with GM2 Gangliosidosis or Niemann-Pick ...
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