30 Participants Needed

Nomlabofusp for Friedreich's Ataxia

TR
KG
Overseen ByKerri Gallagher, RN, B
Age: < 18
Sex: Any
Trial Phase: Phase 1
Sponsor: Larimar Therapeutics, Inc.

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial tests a new treatment called nomlabofusp to determine its safety and tolerability in children and teens with Friedreich's ataxia, a rare genetic disease affecting movement and coordination. Participants will receive either the treatment or a placebo (a harmless substance resembling the treatment) through daily injections for a week. Ideal candidates are those diagnosed with Friedreich's ataxia, who can move at least 25 feet with or without assistance, and manage basic self-care tasks with minimal help. As a Phase 1 trial, this research aims to understand how the treatment works in people, offering participants the opportunity to be among the first to receive it.

Will I have to stop taking my current medications?

The trial does not specify if you need to stop taking your current medications, but you cannot have taken omaveloxolone within 30 days before the trial starts.

Is there any evidence suggesting that nomlabofusp is likely to be safe for humans?

Research has shown that nomlabofusp, a treatment for Friedreich's ataxia, has undergone safety evaluation. In earlier studies, patients generally tolerated nomlabofusp well, with no major safety issues identified, though some side effects may occur, as with any medication. The FDA recommends a safety database with at least 30 participants using the treatment continuously for six months. This requirement indicates that the treatment has been assessed sufficiently for more detailed study. While research continues, these findings offer a positive indication of nomlabofusp's safety in humans.12345

Why do researchers think this study treatment might be promising for Friedreich's ataxia?

Nomlabofusp is unique because it offers a new approach to treating Friedreich's Ataxia through subcutaneous injection, potentially providing more precise dosing and ease of use compared to oral medications. Unlike standard treatments that often focus on managing symptoms, Nomlabofusp may target the underlying causes of the condition more directly. Researchers are excited because this treatment could offer faster relief, with results potentially visible in just seven days, which is quicker than many existing options.

What evidence suggests that nomlabofusp might be an effective treatment for Friedreich's ataxia?

Research shows that nomlabofusp, which participants in this trial may receive, might help treat Friedreich's ataxia by boosting levels of frataxin, a protein typically low in individuals with this condition. Studies have found that after 14 days of treatment, patients experienced a noticeable increase in frataxin levels. Long-term evidence suggests that these protein levels continue to improve. This rise in frataxin is promising because it could address some of the core problems in Friedreich's ataxia.12467

Who Is on the Research Team?

LT

Larimar Therapeutics, Inc.

Principal Investigator

Larimar Therapeutics, Inc.

Are You a Good Fit for This Trial?

This trial is for children and adolescents aged 2 to less than 18 with genetically confirmed Friedreich's ataxia. They must be able to move 25 feet, perform basic self-care, and weigh at least 10 kg. Those with heart conditions or who have taken omaveloxolone recently cannot participate.

Inclusion Criteria

I can walk 25 feet with some help and can sit up, move from bed to chair, and take care of myself.
I weigh at least 10 kilograms.
I am between 2 and 17 years old.
See 1 more

Exclusion Criteria

I have taken omaveloxolone within the last 30 days.
I have been diagnosed with Friedreich's ataxia with a specific genetic feature.
I do not have any health conditions that could risk my safety in the study.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Participants receive a subcutaneous injection of nomlabofusp or placebo once daily for 7 days

1 week
7 visits (in-person)

Follow-up

Participants are monitored for safety and effectiveness after treatment

up to 72 days

What Are the Treatments Tested in This Trial?

Interventions

  • Nomlabofusp
Trial Overview The study tests the safety of a drug called Nomlabofusp (CTI-1601) against a placebo in young patients with Friedreich's Ataxia. It aims to see how well they tolerate the medication over time.
How Is the Trial Designed?
2Treatment groups
Experimental Treatment
Placebo Group
Group I: NomlabofuspExperimental Treatment1 Intervention
Group II: PlaceboPlacebo Group1 Intervention

Find a Clinic Near You

Who Is Running the Clinical Trial?

Larimar Therapeutics, Inc.

Lead Sponsor

Trials
7
Recruited
270+

Published Research Related to This Trial

In a study of 592 patients with genetically confirmed Friedreich's ataxia, earlier disease onset was linked to a higher number of GAA repeats in the frataxin gene, resulting in an earlier onset of symptoms by approximately 2.3 years for every 100 GAA repeats.
The study found that patients with early and intermediate onset experienced a significantly faster decline in ataxia symptoms, as measured by the Scale for the Assessment and Rating of Ataxia (SARA), compared to those with late onset, indicating that age of onset is crucial for understanding disease progression and designing clinical trials.
Biological and clinical characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) cohort: a cross-sectional analysis of baseline data.Reetz, K., Dogan, I., Costa, AS., et al.[2022]
Friedreich's ataxia is linked to a wide range of GAA trinucleotide repeat expansions, with patients showing between 66 to 1360 repeats, and the number of repeats correlates with disease onset and progression.
The study suggests that molecular genetic testing is essential for diagnosing Friedreich's ataxia, as the phenotypic presentation can vary significantly, and some patients may not show the typical symptoms associated with the disorder.
Friedreich's ataxia. Revision of the phenotype according to molecular genetics.Schöls, L., Amoiridis, G., Przuntek, H., et al.[2023]
Friedreich ataxia is a genetic degenerative disease that mainly impacts the nervous system and heart, and its full understanding has evolved since the introduction of genetic testing in 1996.
Research into the complex causes of Friedreich ataxia is aiding the development of new treatments, highlighting the importance of genetic insights in addressing this condition.
Friedreich ataxia.Pandolfo, M.[2008]

Citations

Larimar Therapeutics Announces Positive Data from Ongoing ...In 4 completed studies and the ongoing OL study, 65 participants received at least 1 dose of nomlabofusp, including 39 in the OL study, ...
A Study to Assess Nomlabofusp in Adolescents and ...The goal of this clinical trial is to evaluate the safety and tolerability of nomlabofusp (CTI-1601) in adolescents and children with Friedreich's ataxia (FRDA) ...
Friedreich Ataxia Therapy Nomlabofusp Increases Frataxin ...After 14 days of daily administration, results showed a measurable increase in frataxin concentrations among patients treated with nomlabofusp.
Safety, pharmacokinetics, and pharmacodynamics of ...Results from this study support a favorable safety profile for nomlabofusp. Subcutaneous nomlabofusp injections were quickly absorbed; higher ...
Nomlabofusp leads to long-term frataxin gains for FA patientsTreatment with nomlabofusp is leading to long-term improvements and increased frataxin levels in Friedreich's ataxia patients, new data show.
Larimar Therapeutics Announces FDA Recommendations on ...Larimar Therapeutics Announces FDA Recommendations on Safety Database, and Other Details of Nomlabofusp BLA Submission for Friedreich's Ataxia ...
Press Release: Larimar Therapeutics Announces FDA ...The FDA recommended that the safety database include a total of at least 30 participants with continuous exposure for 6 months including a ...
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