RPS19-encoding lentiviral vector and method of using the same

A self-inactivating lentiviral vector encoding codon-optimized RPS19 addresses the limitations of current Diamond-Blackfan anemia treatments by safely transducing hematopoietic stem cells, alleviating DBAS phenotypes and providing a potential cure with reduced toxicity.

WO2026096338A1 Publication Date: 2026-05-07ST JUDE CHILDRENS RES HOSPITAL INC
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Patent Information

Application Number
PCT/US2025/052592
Authority / Receiving Office
WO · WO
Patent Type
Applications
Current Assignee / Owner
Priority Date
2024-11-04
Filing Date
2025-10-27
Publication Date
2026-05-07

AI Technical Summary

Technical Problem

Current treatments for Diamond-Blackfan anemia syndrome, such as corticosteroids and allogeneic hematopoietic stem cell transplantation, are associated with serious long-term toxicities and suboptimal quality of life, and safe and effective lentiviral vector-based gene therapies are needed to address the hematopoietic defects caused by ribosomal protein S19 mutations.

Method used

A self-inactivating lentiviral vector encoding codon-optimized RPS19 nucleic acids linked to an EFla or MND promoter is developed, devoid of aberrant splice sites, to transduce hematopoietic stem and progenitor cells, providing safe and effective gene therapy for Diamond-Blackfan anemia syndrome.

Benefits of technology

The lentiviral vector effectively alleviates DBAS-like phenotypes in vitro and in vivo, achieving polyclonal integration without clonal dominance or aberrant splicing, offering a potential cure with reduced long-term toxicity.

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Abstract

A self-inactivating lentiviral vector and method for treating or ameliorating Diamond-Blackfan anemia syndrome. The self-inactivating lentiviral vector includes nucleic acids encoding ribosomal protein S19 operably linked to an EF1α promoter or a MND promoter, wherein the nucleic acids encoding RPS19 are codon optimized for expression in human cells and are devoid of aberrant splice acceptor sites, thereby improving the safety and efficacy of the lentiviral vector.
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