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Ilginatinib (NS-018) is an orally bioavailable small molecule inhibitor that selectively targets Janus kinase 2 (JAK2) and Src-family tyrosine kinases. It acts by competitively inhibiting ATP binding to JAK2—including the mutated form JAK2V617F—thereby blocking activation of the JAK/STAT signaling pathway, which is implicated in myeloproliferative disorders such as myelofibrosis. Inhibition of these pathways leads to reduced proliferation and increased apoptosis of malignant hematopoietic cells. Ilginatinib has demonstrated activity in preclinical models and clinical trials for primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis. The drug is under investigation for these indications and has received orphan drug designation from regulatory agencies[1][2][3][4][5][6][8].
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