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GS-100

Phase 3

NGLY1 Deficiency | Gene therapy | Other |Grace Therapeutics, Inc.|Last Updated: May 4, 2026

Success Probability

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Market & Valuation

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Trial Design

CONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment10

FDA Designations

No designations recorded

Clinical trial landscape

GS-100 · 1 trial · 1 indication

Phase 3 1
NCT06199531Safety and Efficacy of GS-100 Gene Therapy in Patients With NGLY1 DeficiencyNGLY1 Deficiency
ACTIVE NOT_RECRUITING10 Analytics
PHASE3ACTIVE NOT_RECRUITING
Safety and Efficacy of GS-100 Gene Therapy in Patients With NGLY1 Deficiency
NGLY1 DeficiencyUnlock trial analytics

Study Endpoints

Primary Endpoints

Phase 1/2 (Dose Finding): Safety and Tolerability of GS-100
Baseline through Week 52

Incidence of adverse events (AEs) and serious AEs (SAEs)

Phase 3 (Pivotal): Efficacy of GS-100 at the Selected Dose
Baseline through Week 52

Improvement in one or more domains of the 88-item Gross Motor Function Measure (GMFM-88) from Baseline to Week 52

Secondary Endpoints

Individual domains of the Bayley Scales of Infant and Toddler Development 4th Ed (BSID-4) for the Cognitive, Language, and Motor scales
Baseline through Week 52
Clinical Global Impression of Change (CGI-C)
Baseline through Week 52
Clinical Global Impression of Severity (CGI-S)
Baseline through Week 52
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Study Design & Arms

AllocationNON_RANDOMIZED
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Cohort 1EXPERIMENTALGS-100 Low Dose Level: 4e14 total vector genomes (vg) for 6-18-year-olds (fully enrolled, 2 participants)
Cohort 2EXPERIMENTALGS-100 Mid Dose Level: 1e15 total vector genomes (vg) for 13-18-year-olds, 8.7e14 total vg for 2-5-year-olds (fully enrolled, 2 participants)
Cohort 3EXPERIMENTALGS-100 High Dose Level: 3e15 total vector genomes (vg) for 6-18-year-olds, 2.6e15 vg for 2-5-year-olds (fully enrolled, 2 participants)
Cohort 4EXPERIMENTALGS-100 Intermediate Dose Level: 2e15 total vector genomes (vg) for 6-18-year-olds, 1.75e15 total vg for 2-5-year-olds (fully enrolled, 1 participant)
Pivotal CohortEXPERIMENTALGS-100 Selected Dose Level: 1e15 total vector genomes (vg) for 6-18-year-olds, 8.7e14 total vg for 2-5-year-olds (fully enrolled, 3 participants)

Interventions

NameTypeDescription
GS-100GENETICA single intracerebroventricular (ICV) dose of GS-100
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Eligibility Criteria

Age Range2 Years to 18 Years
SexALL
Healthy VolunteersNo
Study Sites2

Inclusion Criteria: * Patients must be 2 to 18 years of age, inclusive, at the time of signing the informed consent form (ICF) * Patients with a documented diagnosis of NGLY1 Deficiency based on detection of biallelic variants in the NGLY1 gene via molecular genetic sequencing * Elevated GNA levels...

Countries:United States
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Frequently asked questions about GS-100

What is GS-100 used for?

GS-100 is an investigational gene therapy being developed for the treatment of NGLY1 deficiency, a rare genetic disorder. It is currently in Phase 3 clinical development and has not been approved by the FDA. The therapy is designed to address the underlying cause of the condition.

Who is developing GS-100?

GS-100 is being developed by Grace Therapeutics, Inc., a biopharmaceutical company. The company is conducting clinical trials to evaluate the safety and efficacy of GS-100 in patients with NGLY1 deficiency. Grace Therapeutics is publicly traded under the ticker symbol GRCE.

What phase is GS-100 in?

GS-100 is currently in Phase 3 clinical development for NGLY1 deficiency. It is an investigational gene therapy that has not yet received regulatory approval. The ongoing Phase 3 trial is active but not recruiting participants, indicating that enrollment has been completed.

What clinical trials is GS-100 in?

GS-100 is being evaluated in a Phase 3 clinical trial with the identifier NCT06199531. This trial is assessing the safety and efficacy of GS-100 gene therapy in patients with NGLY1 deficiency. The study is active but not recruiting, with an enrollment target of 10 participants in the United States.

Is GS-100 a gene therapy?

Yes, GS-100 is a gene therapy. It is being developed as a treatment for NGLY1 deficiency, a rare genetic condition. The therapy is currently in Phase 3 clinical trials to evaluate its safety and efficacy in affected patients.