Sena Isadora F G, Prazeres Pedro H D M, Santos Gabryella S P, Borges Isabella T, Azevedo Patrick O, Andreotti Julia P, Almeida Viviani M, Paiva Ana E, Guerra Daniel A P, Lousado Luiza, Souto Luanny, Mintz Akiva, Birbrair Alexander
Department of Pathology, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil.
Department of Radiology, Wake Forest School of Medicine, Winston Salem, NC, USA.
Exp Hematol. 2017 Oct;54:12-16. doi: 10.1016/j.exphem.2017.06.349. Epub 2017 Jul 6.
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.
骨髓纤维化是原发性骨髓纤维化的一个关键组成部分，在原发性骨髓纤维化中，正常骨髓组织和造血细胞逐渐被瘢痕组织所取代。导致骨髓纤维化的具体细胞和分子机制尚不清楚。最近一项使用包括体内谱系追踪在内的先进技术的研究表明，Gli1细胞是导致骨髓纤维化疾病的细胞。令人惊讶的是，Gli1细胞的基因缺失可挽救骨髓衰竭并消除骨髓纤维化。这项工作引入了一个新的骨髓纤维化核心细胞靶点。从这项研究中获得的知识对于治疗多种恶性和非恶性疾病将具有重要意义。
