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LX2020

Phase 1

Arrhythmogenic Cardiomyopathy | Gene therapy | Rare Disease |Lexeo Therapeutics, Inc.|Last Updated: Nov 12, 2025

Target and mechanism

Molecular targetPKP2
ModalityGene therapy

Success Probability

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Market & Valuation

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Trial Design

UNCONTROLLEDDMC
Total Trials1
Total Enrollment10

FDA Designations

FAST_TRACKORPHAN_DRUGRMAT

Clinical trial landscape

LX2020 · 1 trial · 3 indications

Phase 1 1
NCT06109181Gene Therapy for ACM Due to a PKP2 Pathogenic VariantArrhythmogenic Cardiomyopathy
ACTIVE NOT_RECRUITING10 Analytics
PHASE1ACTIVE NOT_RECRUITING
Gene Therapy for ACM Due to a PKP2 Pathogenic Variant
Arrhythmogenic CardiomyopathyUnlock trial analytics

Study Endpoints

Primary Endpoints

Percentage of subjects who experienced at least 1 treatment emergent adverse event (TEAE) and/or 1 treatment emergent serious adverse event (TESAE).
12 months

Evaluation of Safety and Tolerability of LX2020

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Study Design & Arms

AllocationNA
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
LX2020EXPERIMENTALSingle ascending dose of LX2020, with a starting dose of 2.0 x10\^13 gc/kg, in multiple cohorts

Interventions

NameTypeDescription
LX2020GENETICLX2020 is an adeno-associated viral vector encoding the human Plakophilin-2 (PKP2) gene (AAVrh.10hPKP2)
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Eligibility Criteria

Age Range18 Years to 65 Years
SexALL
Healthy VolunteersNo
Study Sites5

Selected Inclusion Criteria: * Adults with a clinical diagnosis of ACM meeting the 2010 revised Task Force Criteria (TFC) * Genetic testing documenting a pathogenic or likely pathogenic variant in PKP2 * Frequent premature ventricular complexes (PVCs) * Implantable cardioverter-defibrillator (ICD) ...

Countries:United States
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Competitive Landscape -Arrhythmogenic Right Ventricular Cardiomyopathy 3 trials (matched to "Arrhythmogenic Cardiomyopathy")

CompanyTickerTrialsLead PhaseDrugs
Lexeo Therapeutics, Inc.LXEO3PHASE1LX2020
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Frequently asked questions about LX2020

What is LX2020 used for?

LX2020 is an investigational gene therapy being developed for arrhythmogenic cardiomyopathy, a rare heart condition. It is designed to address the underlying genetic cause of the disease in patients with a PKP2 pathogenic variant. The therapy is currently in Phase 1 clinical development and has not yet been approved by regulatory authorities.

What does LX2020 target?

LX2020 targets the PKP2 gene, which encodes plakophilin-2, a protein essential for heart muscle cell adhesion. Mutations in PKP2 are a leading cause of arrhythmogenic cardiomyopathy. By delivering a functional copy of the gene, the therapy aims to restore normal protein function and potentially halt disease progression.

Who is developing LX2020?

LX2020 is being developed by Lexeo Therapeutics, Inc., a biopharmaceutical company traded on the Nasdaq under the ticker symbol LXEO. The company specializes in genetic medicines for rare diseases, with a focus on cardiovascular and central nervous system conditions. Lexeo is conducting the ongoing Phase 1 clinical trial for LX2020.

What phase is LX2020 in?

LX2020 is currently in Phase 1 clinical development. It is an investigational drug, meaning it has not been approved by the FDA or any other regulatory agency. The therapy has received Fast Track, Orphan Drug, and Regenerative Medicine Advanced Therapy (RMAT) designations from the FDA, which are intended to expedite its development and review.

What clinical trials is LX2020 in?

LX2020 is being evaluated in a single Phase 1 clinical trial with the identifier NCT06109181. The study, titled 'Gene Therapy for ACM Due to a PKP2 Pathogenic Variant,' is active but not recruiting participants. It is enrolling approximately 10 adults aged 18 years and older in the United States with arrhythmogenic cardiomyopathy caused by a PKP2 mutation.

Is LX2020 the same as PKP2-ARVC therapy?

LX2020 is a gene therapy specifically designed for patients with arrhythmogenic cardiomyopathy caused by a PKP2 pathogenic variant, a condition also referred to as PKP2-ACM or PKP2-ARVC. While the drug is not formally named after the condition, it is intended to treat this specific genetic form of the disease.