Bone marrow fibrosis is a critical component of primary myelofibrosis in which normal bone marrow tissue and blood-forming cells are gradually replaced with scar tissue. The specific cellular and molecular mechanisms that cause bone marrow fibrosis are not understood. A recent study using state-of-the-art techniques, including in vivo lineage tracing, provides evidence that Gli1 cells are the cells responsible for fibrotic disease in the bone marrow. Strikingly, genetic depletion of Gli1 cells rescues bone marrow failure and abolishes myelofibrosis. This work introduces a new central cellular target for bone marrow fibrosis. The knowledge that emerges from this research will be important for the treatment of several malignant and nonmalignant disorders.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6076853 | PMC |
http://dx.doi.org/10.1016/j.exphem.2017.06.349 | DOI Listing |
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