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TSHA-101

Phase 1

Infantile GM2 Gangliosidosis (Disorder) | Monoclonal antibody | Rare Disease |Taysha Gene Therapies, Inc.|Last Updated: May 9, 2023

Target and mechanism

Molecular targetHEXA, HEXB
Target classGenes
ModalityMonoclonal antibody

Success Probability

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Market & Valuation

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Trial Design

UNCONTROLLEDDMC
Total Trials1
Total Enrollment3

FDA Designations

No designations recorded

Clinical trial landscape

TSHA-101 · 1 trial · 1 indication

Phase 1 1
NCT04798235First-in-Human Study of TSHA-101 Gene Therapy for Treatment of Infantile Onset GM2 GangliosidosisInfantile GM2 Gangliosidosis (Disorder)
ACTIVE NOT_RECRUITING3 Analytics
PHASE1ACTIVE NOT_RECRUITING
First-in-Human Study of TSHA-101 Gene Therapy for Treatment of Infantile Onset GM2 Gangliosidosis
Infantile GM2 Gangliosidosis (Disorder)Unlock trial analytics

Study Endpoints

Primary Endpoints

Safety and tolerability: Treatment-emergent Adverse Events (TEAEs)
1 year

Incidence, severity, and relatedness of TEAEs

Safety and Tolerability: Number of participants with abnormal Laboratory assessments
1 year

Number of participants with Changes from Baseline in laboratory assessments

Safety and Tolerability: Electrocardiogram (ECG)
1 year

Changes from Baseline in 12-lead ECG findings in QT interval

Secondary Endpoints

Safety and tolerability: Viral shedding analysis
1 year
Assessment of Immunogenicity: Biomarkers in serum
1 year
Assessment of Immunogenicity: Biomarkers in peripheral blood mononuclear cells (PBMCs
5 years
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Study Design & Arms

AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
TSHA-101EXPERIMENTALSubjects who will receive one-time intrathecal TSHA-101, brain volume based sliding scale for dosage

Interventions

NameTypeDescription
TSHA-101BIOLOGICALAAV9 viral vector containing HEXA and HEXB genes to be administered via Intrathecal injection
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Eligibility Criteria

Age RangeN/A to 15 Months
SexALL
Healthy VolunteersNo
Study Sites1

Key Inclusion Criteria: * male or female with age less than or equal to 15 months * diagnosis of GM2 gangliosidosis with genetic and enzymatic documentation of infantile disease Key Exclusion Criteria: * a second neurodevelopmental disorder independent of the HEXA or HEXB * inability to tolerate ...

Countries:Canada
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Frequently asked questions about TSHA-101

What is TSHA-101 used for?

TSHA-101 is an investigational gene therapy being developed for the treatment of infantile GM2 gangliosidosis, a rare inherited disorder. It is currently in Phase 1 clinical development and has not been approved by regulatory authorities.

What does TSHA-101 target?

TSHA-101 targets the HEXA and HEXB genes, which are involved in the production of enzymes needed to break down GM2 gangliosides. By delivering functional copies of these genes, the therapy aims to address the underlying genetic cause of infantile GM2 gangliosidosis.

Who is developing TSHA-101?

TSHA-101 is being developed by Taysha Gene Therapies, Inc., a biopharmaceutical company. The company is conducting clinical trials to evaluate the safety and efficacy of this investigational therapy.

What phase is TSHA-101 in?

TSHA-101 is in Phase 1 clinical development. It is an investigational drug and has not yet received FDA approval. The ongoing Phase 1 trial is a first-in-human study designed to assess the therapy's safety and preliminary efficacy.

What clinical trials is TSHA-101 in?

TSHA-101 is being studied in a Phase 1 clinical trial with the identifier NCT04798235. This first-in-human study is evaluating the gene therapy for the treatment of infantile onset GM2 gangliosidosis. The trial is active but not recruiting participants.

Is TSHA-101 the same as any other drug?

TSHA-101 is a distinct investigational gene therapy developed by Taysha Gene Therapies. It is not known to be marketed under any other names. Its unique mechanism targets the HEXA and HEXB genes for the treatment of infantile GM2 gangliosidosis.