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Myb-like, SWIRM and MPN domain-containing protein 1 (MYSM1) is a nuclear deubiquitinase and metalloprotease functioning as a chromatin modifier and epigenetic regulator, primarily by removing monoubiquitin from histone H2A, thereby modulating transcription. It plays key roles in hematopoietic stem cell differentiation, B-cell and NK-cell development, and in suppressing excessive innate immune and inflammatory responses by deubiquitinating substrate proteins in cytoplasmic signaling pathways. Dysfunction or mutation leads to bone marrow failure, immunodeficiency, and enhanced inflammation, marking it as both a regulator of gene expression and a therapeutic target in hematological and immune diseases[1][2].
Deubiquitination of monoubiquitinated histone H2A to relieve epigenetic repression. Attenuation of NOD2-mediated inflammation via K63-linked polyubiquitin removal. Inhibition of CGAS-STING pathway by cleavage of K63-linked ubiquitin chains on STING1. Regulation of gene expression involved in hematopoiesis and immune differentiation[2].
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