Two Lead Programs, One Transformative Platform

Global commercial rights for all programs fully owned by Spell Therapeutics.

Program Indication Deficient Enzyme Major Tissues Platform Est. US Patients
Spell-101 Pompe Disease Acid alpha-glucosidase (GAA) Skeletal muscle, heart, lung AAV-mediated ERT ~18,000
Spell-102 Fabry Disease Alpha-galactosidase A (α-GAL) Heart, kidney, nervous system Classical ERT ~29,000
AAV Gene Therapy

Spell-101: Pompe Disease

Pompe disease is caused by a deficiency of acid alpha-glucosidase (GAA), leading to progressive damage in skeletal muscles, heart, and lungs.

Our Spell-101 program uses an AAV vector carrying the IGF2-GAA fusion gene for liver-depot gene therapy — a potential one-time, curative treatment.

The current market leader (Sanofi's Lumizyme/Nexviazyme franchise) generated €316 million in H1 2025, yet patients still face limited efficacy in muscle and lung — precisely where our technology excels.

US Market: ~$1.2B | Global: ~$1.9B

Development Roadmap

Months 0–6: FDA Pre-Meeting

Apply for Fast Track and Rare Disease Designation to accelerate regulatory review.

Months 0–24: Preclinical Studies

In vivo ERT studies in Pompe mice at Duke. Supported by NIH R01 grant (~$2.7M over 5 years).

Years 3–6: IND & Clinical Trials

IND-enabling toxicology studies, FDA filing, GMP vector manufacturing, Phase 1/2 trial initiation.

Classical ERT

Spell-102: Fabry Disease

Fabry disease results from a deficiency of alpha-galactosidase A (α-GAL), causing progressive damage to the heart, kidneys, and nervous system.

Renal failure remains the leading cause of death in Fabry patients, yet no approved ERT effectively treats kidney involvement. Spell-102 aims to be the first ERT to meaningfully address Fabry nephropathy — an $800M+ underserved segment.

Combined Fabry ERT market leaders (Fabrazyme, Replagal, Elfabrio) generated over $1.7 billion in 2024.

US Market: ~$1.4B | Global: ~$2.4B

Development Roadmap

Months 0–6: Pre-IND Meeting

FDA meeting for Fast Track / Rare Disease Designation.

Months 0–30: Preclinical Studies

Efficacy studies in Fabry mice at Duke. GLP-grade enzyme manufacturing with ExcellGene (Switzerland).

Years 3–6: IND & Clinical Trials

IND-enabling studies, FDA filing, GMP enzyme manufacturing, Phase 1/2 clinical trial.

Interested in Our Pipeline?

Learn about the market opportunity behind these programs.