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Primary bone marrow fibrosis (PMF) is a bone marrow growth tumor (MPN) that can lead to sexual fibrosis of bone marrow (BM).
although cell mutations involved in the pathogenesis of PMF have been extensively studied, the sequence events of driving substation activation and fibrosis through hematomatogenic-base cross-linking are still unclear.
using a non-biased method and validating it in MPN patients, the researchers identified spatial differences in the expression of the trending factor CXCL4/plateboard factor 4 (PF4) as a sign of progression of fibrosis.
The lack of hematogenic cell CXCL4 improves MPN dnotypes, reduces substation cell activation and BM fibrosis, and reduces the activation of 3 PMF mouse models: 1) cytonic cell fibrosis path, 2) fibrosis drives cell inflammation, and 3) JAK/STAT activation of cytinocytes and substation cells.
the study shows that in MPN, high expression of CXCL4 has a fibrosis-promoting effect and is a regulatory factor for characteristic inflammation.
, targeting CXCL4 may be an effective way to reduce PMF inflammation.
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