原发性骨髓纤维化（PMF）是发病机制尚未完全明了的少见病。有证据表明造血干细胞（HSC）龛和巨核细胞（MK）稳态失调可促进PMF的发生，并且骨髓树突状细胞（bmDC）缺失导致HSC龛和MK稳态异常。我们前期发现LKB1在bmDC选择性杂合缺失（Zbtb46-Cre LKB1fl/+）导致bmDC显著减少和PMF，推测bmDC缺失LKB1可能破坏了HSC龛和MK的稳态，进而诱发PMF。我们将以PMF的发病机制不明为契机，以DC为主要研究对象，以bmDC在PMF发生发展中的作用和机制为核心科学问题，以LKB1敲除导致稳态下bmDC数量的显著下降并伴随PMF发病的前期实验现象为依据，通过整合多种当前生物学先进研究技术，深入解析LKB1在bmDC的发育、维持和功能中的作用和机制，并探明PMF的致病机理。这将有助于加深人们对bmDC生物学的理解和发现新的PMF致病机制，为PMF防治提供潜在的新靶点。

Primary myelofibrosis (PMF) is a rare disease whose pathogenesis is not yet fully understood. There is evidence that dysregulation of hematopoietic stem cell (HSC) niche and megakaryocyte (MK) homeostasis can promote the development of PMF, and that bone marrow dendritic cell (bmDC) loss leads to abnormal HSC niche and MK homeostasis disorder. Our group generated bmDC-specific LKB1 knockout (Zbtb46-Cre LKB1fl/+) mice, and surprisingly, found that deletion of LKB1 alters bmDC populations and induces PMF. We speculated that bmDC regulates Megakaryocyte-Mesenchymal stromal cell axis to induce PMF. In this study, with modern cellular, molecular immunological methods and transgenic mice strains, we expect to demonstrate the under stream molecular mechanism regulated by the LKB1 signaling in DC, and intend to answer the question how bmDC regulates the occurrence and development in PMF. This might be conducive to better understand the biological characteristics of bmDC and to discover new pathogenic mechanisms, providing a theoretical basis for the development of new means in disease prevention and treatment.