Article ID Journal Published Year Pages File Type
5527479 Experimental Hematology 2017 5 Pages PDF
Abstract

•Bone marrow Gli1+ cells are heterogeneous at their location within the bone marrow.•Gli1+ cells provide a central cellular target in bone marrow fibrosis.•Gli1+ cells act as bone marrow mesenchymal stem cells.

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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