Phase II trial of intravenous human dental pulp stem cell therapy for Huntington’s disease: a randomized, double-blind, placebo-controlled study

Abstract Background Huntington’s disease (HD) is a rare, autosomal dominant neurodegenerative disorder caused by an expansion of cytosine-adenine-guanine (CAG) trinucleotide repeats in the huntingtin (HTT) gene. It manifests with motor, cognitive, and behavioural impairments, leading to progressive...

Full description

Saved in:
Bibliographic Details
Main Authors: Joyce Macedo Sanches Fernandes, Eduardo Pagani, Cristiane Valverde Wenceslau, Leandro Hideki Ynoue, Luciana Ferrara, Irina Kerkis
Format: Article
Language:English
Published: BMC 2025-08-01
Series:Stem Cell Research & Therapy
Subjects:
Online Access:https://doi.org/10.1186/s13287-025-04557-2
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Background Huntington’s disease (HD) is a rare, autosomal dominant neurodegenerative disorder caused by an expansion of cytosine-adenine-guanine (CAG) trinucleotide repeats in the huntingtin (HTT) gene. It manifests with motor, cognitive, and behavioural impairments, leading to progressive functional decline over approximately 20 years. Despite symptomatic treatments, no approved disease-modifying therapies are currently available, though experimental approaches are under investigation. Recent research has explored human dental pulp stem cells (hDPSCs) as a potential therapeutic approach due to their neurotrophic properties and ability to modulate neuroinflammation. This Phase II trial aimed to evaluate the safety and efficacy of NestaCell®, an allogeneic hDPSC-based therapy, in patients with HD. Methods This randomised, double-blind, placebo-controlled trial included 35 patients assigned at a 2:2:1 ratio to receive hDPSCs at 1 million cells/kg, 2 million cells/kg, or placebo over nine intravenous infusions across 11 months. The primary endpoint was the Unified Huntington’s Disease Rating Scale (UHDRS) Total Motor Score (TMS) change. Secondary outcomes included UHDRS Total Functional Capacity (TFC), Total Chorea Score (TCS), Functional Checklist (FC), and magnetic resonance imaging (MRI) based white matter quantification. Safety was assessed by monitoring treatment-emergent adverse events (TEAEs) and laboratory parameters. Results Both doses demonstrated a favourable safety profile, with no increased incidence of adverse events compared to the placebo. No serious adverse event was deemed related to treatment. Both doses significantly improved UHDRS-TMS compared to placebo (p = 0.005), while the 2 million cells/kg group showed significant benefits in UHDRS-TFC (p = 0.011). Additional improvements were observed in the TCS and FC, suggesting a broader clinical impact. MRI analysis indicated a non-significant trend toward neuroprotection, with slower central nervous system (CNS) white and grey matter decline in treated patients. Conclusions NestaCell® was well tolerated and showed statistically significant improvements in motor and functional outcomes in HD patients. While MRI trends suggest a potential neuroprotective effect, further investigation is warranted. These findings support the advancement to a Phase III trial to confirm efficacy and long-term safety in a larger cohort. Trial registration: This study was registered on August 16, 2017, at ClinicalTrials.gov (identifier: NCT03252535; https://clinicaltrials.gov/search?cond=NCT03252535 ).
ISSN:1757-6512