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AMT-130

Phase 1

Huntington Disease | Gene therapy | Neurology |uniQure N.V.|Last Updated: Oct 21, 2025

Target and mechanism

ModalityGene therapy

Also known as intra-striatal rAAV5-miHTT

Success Probability

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Market & Valuation

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Trial Design

RandomizedCONTROLLEDDMC
Total Trials1
Total Enrollment14

FDA Designations

ACCELERATED_APPROVALRMATBREAKTHROUGH_THERAPYFAST_TRACKORPHAN_DRUG

Clinical trial landscape

AMT-130 · 2 trials · 2 indications

Phase 1 2
NCT05243017Safety and Efficacy of AMT-130 in European Adults With Early Manifest Huntington's DiseaseHuntington Disease
ACTIVE NOT_RECRUITING14 Analytics
NCT04120493Safety and Proof-of-Concept (POC) Study With AMT-130 in Adults With Early Manifest Huntington's DiseaseHuntington's Disease
ACTIVE NOT_RECRUITING43 Analytics
PHASE1ACTIVE NOT_RECRUITING
Safety and Efficacy of AMT-130 in European Adults With Early Manifest Huntington's Disease
Huntington DiseaseUnlock trial analytics
PHASE1ACTIVE NOT_RECRUITING
Safety and Proof-of-Concept (POC) Study With AMT-130 in Adults With Early Manifest Huntington's Disease
Huntington's DiseaseUnlock trial analytics

Study Endpoints

Primary Endpoints

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by Adverse Events
6 months

Evaluation will be assessed by; \- Type and incidence of Adverse Events (AEs)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by Vital Signs - Blood Pressure
6 months

Evaluation will be assessed by; \- Changes from baseline in blood pressure (mmHg)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by Vital Signs - Respiratory Rate
6 months

Evaluation will be assessed by; \- Changes from baseline in respiratory rate (BPM)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by Vital Signs - Heart Rate
6 months

Evaluation will be assessed by; \- Changes from baseline in heart rate (BPM)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by Electrocardiograms
6 months

Evaluation will be assessed by; \- Changes from baseline in electrocardiograms (ECGs) for any clinically significant abnormalities or clinically significant worsening. (normal or abnormal)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by changes documented in the neurological examinations
6 months

Evaluation will be assessed by; \- Changes from baseline in neurological examinations including mental status, cranial nerves, sensory, motor, fine motor, reflexes, and gait (normal or abnormal)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by changes documented in the physical examinations
6 months

Evaluation will be assessed by; \- Changes from baseline in physical examinations assessed by physical appearance, HEENT, Neck, Chest and Lungs, Cardiovascular, Abdomen, Musculoskeletal, and Genitourinary (normal or abnormal)

Evaluate the safety and tolerability by number of participants with clinically significant changes in laboratory tests - Clinical Chemistry
6 months

Evaluation will be assessed by; \- Changes from baseline in Clinical Chemistry laboratory tests with clinical significance.

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by number of participants with clinically significant laboratory tests - hematology
6 months

Evaluation will be assessed by; \- Changes from baseline in hematology laboratory tests with clinical significance.

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by number of participants with clinical significant laboratory tests - urinalysis
6 months

Evaluation will be assessed by; \- Change from baseline in routine urinalysis test with clinical significance.

Evaluate the safety and tolerability o by number of participants with clinical significant changes in cerebrospinal fluid (CSF) analysis
6 months

Evaluation will be assessed by; \- Change from baseline in CSF analysis with clinical significance.

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by vector shedding
6 months

Evaluation will be assessed by; \- Change over time in AAV5 vector shedding

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by laboratory testing serum and CSF for biomarkers
6 months

Evaluation will be assessed by; \- Change over time in microglial activation (YKL-40) (pg/mL)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by cognitive assessment
6 months

Evaluation will be assessed by; \- Change from baseline to Day 14 and Month 1 in the Montreal Cognitive Assessment (MoCA)

Evaluate the safety and tolerability of bilateral striatal delivery of AMT-130 as a total HTT gene lowering therapy in adult subjects with early manifest HD assessed by number of participants with changes in MRI
6 months

Evaluation will be assessed by; \- Change from baseline will be measured by edema, inflammation, volume loss, and structural changes as measured by the following MRI pulse sequences, T1, T2 and diffusion MRI (dMRI)

Number and type of Adverse Events (AE)
12 months (Cohorts 1 & 2) and 12 months (Cohort 3)

Safety will be assessed by adverse events (AEs) related to clinical safety laboratory tests, vital signs, electrocardiograms (ECGs), neurological and physical examinations, rAAV5 vector shedding, immunogenicity response (Cohorts 1, 2 \& 3), suicidality risk \[Columbia-Suicide Severity Rating Scale \[C-SSRS)\], changes in global cognitive functioning \[Montreal Cognitive Assessment Scale (MoCA)\] and MRI measures of edema, inflammation, volume loss and structural changes.

Secondary Endpoints

Duration of persistence of AMT-130 in the brain
Collected for duration of study through month 60
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Study Design & Arms

AllocationRANDOMIZED
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Cohort 1EXPERIMENTALLow dose AMT-130 (6 × 10\^12 gc/subject)
Cohort 2EXPERIMENTALHigh dose AMT-130 (6 × 10\^13 gc/subject)
Cohort 3EXPERIMENTALLow dose AMT-130 (6 × 10\^12 gc/subject) High dose AMT-130 (6 × 10\^13 gc/subject)
Cohorts 1, 2SHAM_COMPARATORImitation (sham) surgery
Cohort 4EXPERIMENTALHigh dose rAAV5-miHTT (6x10\^13 gc/subject).

Interventions

NameTypeDescription
intra-striatal rAAV5-miHTTGENETICOne time MRI-guided stereotaxic infusion of rAAV5-miHTT into the brain
Imitation (sham) surgeryOTHERSimulated surgical procedure with skin incisions only; no intrastriatal injections and no burr holes through the skull
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Eligibility Criteria

Age Range25 Years to 65 Years
SexALL
Healthy VolunteersNo
Study Sites4

Inclusion Criteria: 1. Able and willing to provide written informed consent prior to the study and study-related procedure. 2. Male and female participants 25-65 years of age. 3. Cohorts 1 \& 2: 1. a DCL of 4 OR 2. a DCL of 3 with either a positive ("Yes") response to the UHDRS Question 80 (...

Countries:PolandUnited KingdomUnited States
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Competitive Landscape -Huntington's Disease 8 trials (matched to "Huntington Disease")