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LX2006

Phase 2

Friedreich Ataxia | Gene therapy | Rare Disease |Lexeo Therapeutics, Inc.|Last Updated: Sep 2, 2026

Target and mechanism

Molecular targetFXN
ModalityGene therapy

Also known as Low dose LX2006, Mid Dose LX2006

Success Probability

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Market & Valuation

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Trial Design

RandomizedCONTROLLEDDMC
Total Trials2
Total Enrollment34

FDA Designations

BREAKTHROUGH_THERAPYRMATORPHAN_DRUGFAST_TRACKRARE_PEDIATRIC_DISEASEACCELERATED_APPROVAL

Clinical trial landscape

LX2006 · 2 trials · 2 indications

Phase 2 1Phase 1 1
NCT07721025Study of LX2006 Gene Therapy in Friedreich Ataxia CardiomyopathyFriedreich Ataxia
RECRUITING26 Analytics
PHASE2RECRUITING
Study of LX2006 Gene Therapy in Friedreich Ataxia Cardiomyopathy
Friedreich AtaxiaUnlock trial analytics

Study Endpoints

Primary Endpoints

Cohort 1: Percent change from baseline in left ventricular mass index by cardiac MRI
At Week 26
Cohort 2: Incidence and severity of treatment-emergent adverse events
Through Month 60
Cohort 2: Change in clinical routine safety laboratory tests as assessed by standardized clinical laboratory reference ranges
Through Month 60
Cohort 2: Change in clinical vital signs (body temperature [°C])
Through Month 60
Cohort 2: Change in clinical vital signs (systolic and diastolic blood pressure [mmHg])
Through Month 60
Cohort 2: Change in clinical vital signs (heart rate [beats per minute])
Through Month 60
Cohort 2: Change in clinical vital signs (respiratory rate [breaths per minute])
Through Month 60
Cohort 2: Change in clinical vital signs (oxygen saturation [%])
Through Month 60
Cohort 2: Change in clinical 12-lead ECG findings (ECG machine automatically calculates the heart rate [beats per minute] and measures PR, QT, QT interval corrected using Fridericia's method intervals, and QRS complex [milliseconds])
Through Month 60
Treatment-emergent adverse events (TEAEs) and Treatment-emergent serious events (TESAEs)
Change from baseline to end of year 5 post dose

Secondary Endpoints

Cohorts 1 and 2: Change from baseline in high-sensitivity troponin I
At Week 26
Cohort 1: Change from baseline in maximal wall thickness by cardiac MRI
At Week 26
Cohort 1: Number of the following cardiovascular events as collected from the participant by the study doctor during scheduled study visits
At Month 60
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Study Design & Arms

AllocationRANDOMIZED
MaskingSINGLE
ModelCROSSOVER
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
LX2006EXPERIMENTALCohort 1: Participants ≥16 years of age with FA-CM Cohort 2: Participants ≥6 to \<16 years of age with FA-CM. As Cohort 2 will not be randomized, all participants will receive LX2006 upon enrollment. Participants in Cohort 2 will be enrolled after safety is assessed in a group of participants in Cohort 1.
Usual CareOTHERCohort 1: Participants ≥16 years of age with FA-CM Participants will receive usual care for 26 weeks before receiving treatment with LX2006 (single crossover).
Cohort 1/ Cohort 2/ Cohort 3EXPERIMENTAL -

Interventions

NameTypeDescription
LX2006GENETICAdeno-associated viral vector encoding the FXN gene (AAVrh.10hFXN)
Usual CareOTHERCohort 1: Participants ≥16 years of age with FA-CM Participants will receive usual care for 26 weeks before receiving treatment with LX2006 (single crossover).
Low dose LX2006GENETICAdeno-associated viral vector encoding the FXN gene (AAVrh.10hFXN)
Mid Dose LX2006GENETICAdeno-associated viral vector encoding the FXN gene (AAVrh.10hFXN)
High Dose LX2006GENETICAdeno-associated viral vector encoding the FXN gene (AAVrh.10hFXN)
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Eligibility Criteria

Age Range6 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites1

Inclusion Criteria: * Male or female, age at least 6 years at the time of signing the informed consent (and assent, if applicable). * Diagnosis of FA, based on clinical phenotype and genotype (GAA expansion on the frataxin gene) * Onset of FA on or before 25 years of age * Confirmed left ventricula...

Countries:United States
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Recent Changes (Last 90 Days)

LOWSep 2, 2026NCT07721025lastUpdatePostDate: changed
LOWSep 2, 2026NCT07721025lastUpdatePostDate: changed
LOWSep 2, 2026NCT07721025lastUpdatePostDate: changed
LOWJul 22, 2026NCT07721025NEW_TRIAL: changed
LOWJul 22, 2026NCT07721025NEW_TRIAL: changed

Frequently asked questions about LX2006

What is low dose LX2006 used for in Friedreich Ataxia?

Low dose LX2006 is an investigational gene therapy being developed for Friedreich Ataxia, specifically for cardiomyopathy associated with the condition. It is designed to address the cardiac complications of Friedreich Ataxia and is currently in clinical development, with a Phase 2 trial recruiting patients.

What does LX2006 target?

LX2006 targets the FXN gene, which encodes the frataxin protein. In Friedreich Ataxia, mutations in FXN lead to frataxin deficiency, and LX2006 is designed to deliver a functional copy of the gene to address the underlying cause of the disease, particularly its cardiac manifestations.

Who makes LX2006?

LX2006 is developed by Lexeo Therapeutics, Inc., a biopharmaceutical company traded on Nasdaq under the ticker LXEO. The company is advancing LX2006 as a gene therapy for Friedreich Ataxia cardiomyopathy.

What phase is LX2006 in?

LX2006 is in Phase 2 clinical development for Friedreich Ataxia cardiomyopathy. A Phase 2 trial (NCT07721025) is currently recruiting, and a Phase 1 trial (NCT05445323) is active but not recruiting. The therapy has received FDA designations including Breakthrough Therapy, RMAT, Orphan Drug, Fast Track, Rare Pediatric Disease, and Accelerated Approval.

What clinical trials is LX2006 in?

LX2006 is being studied in two clinical trials. NCT07721025 is a Phase 2 study in Friedreich Ataxia cardiomyopathy, recruiting 26 participants aged 6 years and older in the United States. NCT05445323 is a Phase 1 study in the same condition, with 8 participants aged 18 and older, and is active but not recruiting.

Is LX2006 the same as LX2006?

Yes, low dose LX2006 is the same drug as LX2006. The term low dose refers to a specific dosage level being evaluated in clinical trials, but the drug asset itself is LX2006, developed by Lexeo Therapeutics for Friedreich Ataxia cardiomyopathy.