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NGN-101

Phase 1

Neuronal Ceroid Lipofuscinosis CLN5 | Gene therapy | Rare Disease |Neurogene Inc.|Last Updated: Aug 12, 2024

Success Probability

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Market & Valuation

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Trial Design

CONTROLLEDDMC
Total Trials1
Total Enrollment6

FDA Designations

No designations recorded

Clinical trial landscape

NGN-101 · 1 trial · 1 indication

Phase 1 1
NCT05228145Gene Therapy Study for Children With CLN5 Batten DiseaseNeuronal Ceroid Lipofuscinosis CLN5
ACTIVE NOT_RECRUITING6 Analytics
PHASE1ACTIVE NOT_RECRUITING
Gene Therapy Study for Children With CLN5 Batten Disease
Neuronal Ceroid Lipofuscinosis CLN5Unlock trial analytics

Study Endpoints

Primary Endpoints

Incidence of Treatment Emergent Adverse Events (TEAEs)
5 years (multiple visits)

Incidence, type, severity, and frequency of TEAEs

Incidence of Serious Adverse Events (SAEs)
5 years (multiple visits)

Incidence, type, severity, and frequency of SAEs

Incidence of clinical laboratory abnormalities
5 years (multiple visits)

Incidence, type, severity, and frequency of clinical laboratory abnormalities

Incidence of new nerve conduction study (NCS) abnormalities
5 years (multiple visits)

Incidence, type, severity, and frequency of new nerve conduction study (NCS) abnormalities

Incidence of new physical and neurologic exam abnormalities
5 years (multiple visits)

Incidence, type, severity, and frequency of new physical and neurologic exam abnormalities

Secondary Endpoints

Change in Hamburg Scale, Motor and Language domain scores
5 years (multiple visits)
Change in Spectral Domain-Optical Coherence Tomography (SD-OCT)
5 years (multiple visits)
Change in Unified Batten Diseases Rating Scale (UBDRS)
5 years (multiple visits)
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Study Design & Arms

AllocationNON_RANDOMIZED
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Cohort 1EXPERIMENTALThe study treatment is a recombinant serotype 9 adeno-associated virus encoding a codon-optimized human CLN5 transgene (hCLN5opt).
Cohort 2EXPERIMENTALThe study treatment is a higher dose of recombinant serotype 9 adeno-associated virus encoding a codon-optimized human CLN5 transgene (hCLN5opt).
Cohort 3EXPERIMENTALThe study treatment is a higher dose of recombinant serotype 9 adeno-associated virus encoding a codon- optimized human CLN5 transgene (hCLN5opt).

Interventions

NameTypeDescription
NGN-101GENETICParticipants with confirmed mutations in the CLN5 gene who meet all the inclusion and none of the exclusion criteria will be treated with a single intracerebroventricular (ICV) dose and a single intravitreal (IVT) dose of the study treatment.
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Eligibility Criteria

Age Range3 Years to 9 Years
SexALL
Healthy VolunteersNo
Study Sites2

Inclusion Criteria * Age from 3 to 9 years (Child) * Molecular genetic diagnosis of the CLN5 gene * Confirmed clinical diagnosis of CLN5 disease * Impaired motor and/or language function and/or impaired visual acuity * Written informed consent from parent or legal guardian and assent from study par...

Countries:United StatesUnited Kingdom
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Frequently asked questions about NGN-101

What is NGN-101 used for?

NGN-101 is an investigational gene therapy being developed for Neuronal Ceroid Lipofuscinosis CLN5, a rare genetic disorder also known as CLN5 Batten disease. It is designed to address the underlying genetic cause of this condition, which primarily affects the nervous system in children.

Who makes NGN-101?

NGN-101 is being developed by Neurogene Inc., a biopharmaceutical company focused on rare neurological diseases. Neurogene is publicly traded on the Nasdaq under the ticker symbol NGNE.

What phase is NGN-101 in?

NGN-101 is currently in Phase 1 clinical development. It is an investigational therapy, meaning it has not been approved by regulatory authorities and is still being studied for safety and efficacy in clinical trials.

What clinical trials is NGN-101 in?

NGN-101 is being evaluated in a Phase 1 clinical trial with the identifier NCT05228145, titled 'Gene Therapy Study for Children With CLN5 Batten Disease.' The trial is active but not recruiting, with an enrollment of 6 participants, and is being conducted in the United States and United Kingdom.

How does NGN-101 work?

NGN-101 is a gene therapy designed to deliver a functional copy of the CLN5 gene to cells. This approach aims to correct the genetic deficiency that causes CLN5 Batten disease, potentially slowing or halting disease progression. The therapy is administered to children aged 3 years and older.