BRAFV600E induces key features of LCH in iPSCs with cell type-specific phenotypes and drug responses.

doi:10.1182/blood.2024026066

Article

BRAFV600E induces key features of LCH in iPSCs with cell type-specific phenotypes and drug responses.

Blood - 20 Feb 2025

Abagnale Giulio, Schwentner Raphaela, Ben Soussia-Weiss Philipp, van Midden Wouter, Sturtzel Caterina, Pötschger Ulrike, Rados Magdalena, Taschner-Mandl Sabine, Simonitsch-Klupp Ingrid, Hafemeister Christoph, Halbritter Florian, Distel Martin, Eder Sebastian K, Hutter Caroline

Abstract excerpt

ABSTRACT: Langerhans cell histiocytosis (LCH) is a clonal hematopoietic disorder defined by tumorous lesions containing CD1a+/CD207+ cells. Two severe complications of LCH are systemic hyperinflammation and progressive neurodegeneration. The scarcity of primary samples and lack of appropriate models limit our mechanistic understanding of LCH pathogenesis and affect patient care. We generated a human in vitro...

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Topics

Humans
Induced Pluripotent Stem Cells
Proto-Oncogene Proteins B-raf
Histiocytosis, Langerhans-Cell

BRAFV600E induces key features of LCH in iPSCs with cell type-specific phenotypes and drug responses.

Abstract excerpt

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