An Open Label Extension Study of CTI-1601 in Subjects With Friedreich's Ataxia

NCT ID: NCT06447025

Last Updated: 2024-06-06

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

ENROLLING_BY_INVITATION

Clinical Phase

PHASE2

Total Enrollment

75 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-01-25

Study Completion Date

2027-01-31

Brief Summary

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This is an open-label extension (OLE) study designed to evaluate the long-term safety, tolerability, pharmacokinetics (PK), pharmacodynamics (PD), and clinical effects of subcutaneous (SC) administration of CTI-1601, also known as nomlabofusp, in subjects with Friedreich's ataxia (FRDA).

The objectives of this OLE study are:

* To evaluate the safety of long-term subcutaneous (SC) administration of CTI-1601 in subjects with FRDA
* To evaluate the PK of long-term subcutaneous (SC) administration of CTI-1601 in subjects with FRDA
* To evaluate the effect of long-term subcutaneous (SC) administration of CTI-1601 in subjects with FRDA on:

* Tissue FXN concentrations
* Clinical evaluations of FRDA
* Gene Expression and select lipids

Detailed Description

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This is an open label extension (OLE) study in patients with FRDA who participated in a prior clinical study of CTI-1601 to evaluate the safety, PK, PD, and clinical effects of long-term daily administration of CTI-1601.

Conditions

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Friedreich Ataxia

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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CTI-1601

CTI-1601 will be administered daily

Group Type EXPERIMENTAL

CTI-1601

Intervention Type BIOLOGICAL

CTI-1601 is a recombinant fusion protein and is intended to deliver human frataxin, the protein deficient in Friedreich's ataxia

Interventions

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CTI-1601

CTI-1601 is a recombinant fusion protein and is intended to deliver human frataxin, the protein deficient in Friedreich's ataxia

Intervention Type BIOLOGICAL

Other Intervention Names

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Nomlabofusp

Eligibility Criteria

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Inclusion Criteria

* Subjects with FRDA who previously completed participation in a study of CTI-1601 will be eligible to participate in this study unless the subject experienced one or more of the following in a previous CTI-1601 study: a) serious adverse event (SAE) related to study drug; b) significant AE, defined as Grade 3 or higher according to the Common Terminology Criteria for Adverse Events (CTCAE), version 5.0 (or higher), related to study drug; c) some other event, related to participation in a previous study with CTI-1601, that supports the exclusion of the subject from participating in this study as determined by the Sponsor (i.e., an AE considered clinically significant by the Sponsor regardless of whether it met SAE criteria and regardless of CTCAE grade); d) Withdraw from participation in a previous study of CTI-1601 for any reason.
* Subject has a HbA1c less than or equal to 7.0%.
* Subject must demonstrate sufficient dexterity and visual acuity to prepare and self-administer SC injections of CTI-1601 QD or is able to identify a caregiver who will be trained and committed to prepare and administer the daily injections.

Exclusion Criteria

* Subjects who are confirmed as compound heterozygous (GAA repeat expansion on only one allele) for FRDA.
* Subject has any condition, disease, or situation, including a cardiac condition or disease, that in the opinion of the PI, could confound the results of the study or put the subject at undue risk, making participation inadvisable.
* Subject used any investigational drug (other than CTI-1601) or device within 90 days prior to Screening.
* Subject requires use of amiodarone.
* Subject used erythropoietin, etravirine, or gamma interferon within 90 days prior to Screening.
* Subject use of biotin supplementation that exceeds 30 mcg/day, either as part of a multivitamin or as a standalone supplement, within 7 days prior to the first dose of study drug. Biotin supplementation ≤30 mcg/day is permitted if taken at a stable dose and frequency for at least 28 days prior to Screening and there is a commitment from the subject to maintain the biotin dose throughout the study (due to interference with assays).
* Subject uses more than 3 grams of acetaminophen daily.
* Subject receives medication that requires SC injection in the abdomen or thigh.
* Subject is unable to discontinue medications that have not been at a stable dose and frequency for at least 28 days prior to Screening.
* Subject has a Screening echocardiogram (ECHO) LVEF \< 45%.
* Male subject has a QTcF \> 450 milliseconds or female subject has a QTcF \> 470 milliseconds on an ECG.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Larimar Therapeutics, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Larimar Therapeutics, Inc.

Role: STUDY_CHAIR

Larimar Therapeutics, Inc.

Locations

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University of California Los Angeles

Los Angeles, California, United States

Site Status

Fixel Institute for Neurological Disease, University of Florida Health

Gainesville, Florida, United States

Site Status

Morsani Center for Advanced Health Care, University of South Florida Health

Tampa, Florida, United States

Site Status

University of Iowa

Iowa City, Iowa, United States

Site Status

Clinilabs Drug Development, Corp.

Eatontown, New Jersey, United States

Site Status

Ohio State University United States

Columbus, Ohio, United States

Site Status

Hospital of the University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status

Countries

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United States

References

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Delatycki MB, Corben LA. Clinical features of Friedreich ataxia. J Child Neurol. 2012 Sep;27(9):1133-7. doi: 10.1177/0883073812448230. Epub 2012 Jun 29.

Reference Type BACKGROUND
PMID: 22752493 (View on PubMed)

Deutsch EC, Santani AB, Perlman SL, Farmer JM, Stolle CA, Marusich MF, Lynch DR. A rapid, noninvasive immunoassay for frataxin: utility in assessment of Friedreich ataxia. Mol Genet Metab. 2010 Oct-Nov;101(2-3):238-45. doi: 10.1016/j.ymgme.2010.07.001. Epub 2010 Jul 8.

Reference Type BACKGROUND
PMID: 20675166 (View on PubMed)

Fahey MC, Corben L, Collins V, Churchyard AJ, Delatycki MB. How is disease progress in Friedreich's ataxia best measured? A study of four rating scales. J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):411-3. doi: 10.1136/jnnp.2006.096008. Epub 2006 Oct 20.

Reference Type BACKGROUND
PMID: 17056635 (View on PubMed)

Fisk JD, Ritvo PG, Ross L, Haase DA, Marrie TJ, Schlech WF. Measuring the functional impact of fatigue: initial validation of the fatigue impact scale. Clin Infect Dis. 1994 Jan;18 Suppl 1:S79-83. doi: 10.1093/clinids/18.supplement_1.s79.

Reference Type BACKGROUND
PMID: 8148458 (View on PubMed)

Goodkin DE, Hertsgaard D, Seminary J. Upper extremity function in multiple sclerosis: improving assessment sensitivity with box-and-block and nine-hole peg tests. Arch Phys Med Rehabil. 1988 Oct;69(10):850-4.

Reference Type BACKGROUND
PMID: 3178453 (View on PubMed)

Koeppen AH. Friedreich's ataxia: pathology, pathogenesis, and molecular genetics. J Neurol Sci. 2011 Apr 15;303(1-2):1-12. doi: 10.1016/j.jns.2011.01.010.

Reference Type BACKGROUND
PMID: 21315377 (View on PubMed)

Guidelines MSCfCP. Fatigue and multiple sclerosis: evidence-based management strategies for fatigue in multiple sclerosis. 1998.

Reference Type BACKGROUND

Lazaropoulos M, Dong Y, Clark E, Greeley NR, Seyer LA, Brigatti KW, Christie C, Perlman SL, Wilmot GR, Gomez CM, Mathews KD, Yoon G, Zesiewicz T, Hoyle C, Subramony SH, Brocht AF, Farmer JM, Wilson RB, Deutsch EC, Lynch DR. Frataxin levels in peripheral tissue in Friedreich ataxia. Ann Clin Transl Neurol. 2015 Aug;2(8):831-42. doi: 10.1002/acn3.225. Epub 2015 Jul 1.

Reference Type BACKGROUND
PMID: 26339677 (View on PubMed)

Pandolfo M. Friedreich ataxia. Arch Neurol. 2008 Oct;65(10):1296-303. doi: 10.1001/archneur.65.10.1296.

Reference Type BACKGROUND
PMID: 18852343 (View on PubMed)

Rudick R, Antel J, Confavreux C, Cutter G, Ellison G, Fischer J, Lublin F, Miller A, Petkau J, Rao S, Reingold S, Syndulko K, Thompson A, Wallenberg J, Weinshenker B, Willoughby E. Clinical outcomes assessment in multiple sclerosis. Ann Neurol. 1996 Sep;40(3):469-79. doi: 10.1002/ana.410400321.

Reference Type BACKGROUND
PMID: 8797541 (View on PubMed)

Wang C, Lu N, Chen WC, Li H, Tiwari R, Xu Y, Yue LQ. Propensity score-integrated composite likelihood approach for incorporating real-world evidence in single-arm clinical studies. J Biopharm Stat. 2020 May 3;30(3):495-507. doi: 10.1080/10543406.2019.1684309. Epub 2019 Nov 10.

Reference Type BACKGROUND
PMID: 31707908 (View on PubMed)

Other Identifiers

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CLIN-1601-201

Identifier Type: -

Identifier Source: org_study_id

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