Deubiquitinase catalytic activity of MYSM1 is essential in vivo for hematopoiesis and immune cell development.

ABSTRACT: Myb-like SWIRM and MPN domains 1 (MYSM1) is a chromatin binding protein with deubiquitinase (DUB) catalytic activity. Rare MYSM1 mutations in human patients result in an inherited bone marrow failure syndrome, highlighting the biomedical significance of MYSM1 in the hematopoietic system. We and others characterized Mysm1-knockout mice as a model of this disorder and established that MYSM1 regulates hematopoietic function and leukocyte development in such models through different mechanisms. It is, however, unknown whether the DUB catalytic activity of MYSM1 is universally required for its many functions and for the maintenance of hematopoiesis in vivo. To test this, here we generated a new mouse strain carrying a Mysm1^D660N point mutation (Mysm1^DN) and demonstrated th

SUBMITTER: Liang Y 

PROVIDER: S-EPMC9825392 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature