A Randomized Study of SPK-10001 Gene Therapy in Participants With Huntington's Disease

NCT ID: NCT06826612

Last Updated: 2025-12-17

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

RECRUITING

Clinical Phase

PHASE1/PHASE2

Total Enrollment

53 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-02-21

Study Completion Date

2035-01-12

Brief Summary

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The main goal of this study is to evaluate the safety, tolerability, and preliminary efficacy of SPK-10001 in participants with Huntington's Disease.

Detailed Description

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Conditions

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Huntington Disease

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

SEQUENTIAL

The study will be conducted in 4 sequential parts.

* Part A is an open-label cohort in which all participants will receive SPK-10001.
* Part B is randomized, double-blind and placebo-surgery-controlled.
* Part C is a crossover open-label portion where participants who received placebo-surgery control in Part B will receive SPK-10001.
* Part D is long term follow-up after completion of active treatment in any of Parts A, B, or C.
Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors
* Part A is open-label and non-randomized.
* Part B is blinded and randomized.
* Part C is open-label and non-randomized.
* Part D is open-label and non-randomized.

Study Groups

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SPK-10001

Group Type EXPERIMENTAL

SPK-10001

Intervention Type GENETIC

Specified dose on specified days

Placebo Surgery Control

Group Type OTHER

Placebo Surgery Control

Intervention Type OTHER

Placebo Surgery procedure for SPK-10001

Interventions

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SPK-10001

Specified dose on specified days

Intervention Type GENETIC

Placebo Surgery Control

Placebo Surgery procedure for SPK-10001

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Have confirmed huntingtin (HTT) cytosine-adenine-guanine (CAG) repeat length ≥40 on genetic testing and confirmation diagnostic test by the central laboratory (CL) at screening.
* Have striatal atrophy demonstrated by caudate/intracranial volume less than the age-adjusted cutoff values associated with HDISS Stage 1.
* Have UHDRS Total Motor Score (TMS) equal to or greater than the age-adjusted cutoff value associated with HDISS Stage 2.
* Have UHDRS Total Functional Capacity (TFC) greater than or equal to 11.
* Use of cholinesterase inhibitors, memantine, amantadine, or riluzole must have been at stable dosing for at least 12 weeks before screening and baseline and anticipated to remain stable during the first 12 months after SPK-10001 administration.
* Antidepressant or benzodiazepine use must have been at stable dosing for at least 12 weeks before screening and baseline and anticipated to remain stable during the first 12 months after SPK-10001 administration.
* Antipsychotics for motor symptoms or mood stabilization (i.e., irritability or aggressive behavior) and/or tetrabenazine, valbenazine, or deutetrabenazine must have been at a stable dose for at least 12 weeks before screening and baseline and are anticipated to remain stable during the first 12 months after SPK-10001 administration.

Exclusion Criteria

* A safe trajectory is not able to be identified for targeting placement of the cannula into the caudate or putamen on both sides of the brain due to extent of atrophy or other anatomical features.
* Have received an antisense oligonucleotide therapy during the past year.
* History of deep brain stimulation.
* History of or intention to undergo gene therapy, cell transplantation, or brain surgery during the course of the study.
* Have participated in an investigational drug study with a systemic administration within 6 weeks or 5 half-lives of screening, whichever is longer.
Minimum Eligible Age

25 Years

Maximum Eligible Age

65 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Hoffmann-La Roche

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Beth Israel Deaconess Medical Center

Boston, Massachusetts, United States

Site Status RECRUITING

University of Cincinnati/Cincinnati Children's Hospital

Cincinnati, Ohio, United States

Site Status RECRUITING

Ohio State University

Columbus, Ohio, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Huntington Study Group (HSG)

Role: CONTACT

800-487-7671

Clinical Trial Director

Role: CONTACT

Spark Therapeutics, Inc.

Facility Contacts

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Dr. Samuel Frank, MD

Role: primary

(617) 667-2355

Clementina Ullman

Role: backup

(617) 667-2355

Andrew Duker, MD

Role: primary

Lauren Wingler

Role: backup

513-558-0222

Sandra Kostyk, MD, PhD

Role: primary

Nicole Vrettos

Role: backup

614-366-8642

Other Identifiers

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SPK-10001-101

Identifier Type: -

Identifier Source: org_study_id