Kenner Lukas, Hoebertz Astrid, Beil F Timo, Keon Niamh, Karreth Florian, Eferl Robert, Scheuch Harald, Szremska Agnieszka, Amling Michael, Schorpp-Kistner Marina, Angel Peter, Wagner Erwin F
Research Institute of Molecular Pathology, Dr. Bohr-Gasse 7, A-1030 Vienna, Austria.
J Cell Biol. 2004 Feb 16;164(4):613-23. doi: 10.1083/jcb.200308155. Epub 2004 Feb 9.
Because JunB is an essential gene for placentation, it was conditionally deleted in the embryo proper. JunBDelta/Delta mice are born viable, but develop severe low turnover osteopenia caused by apparent cell-autonomous osteoblast and osteoclast defects before a chronic myeloid leukemia-like disease. Although JunB was reported to be a negative regulator of cell proliferation, junBDelta/Delta osteoclast precursors and osteoblasts show reduced proliferation along with a differentiation defect in vivo and in vitro. Mutant osteoblasts express elevated p16(INK4a) levels, but exhibit decreased cyclin D1 and cyclin A expression. Runx2 is transiently increased during osteoblast differentiation in vitro, whereas mature osteoblast markers such as osteocalcin and bone sialoprotein are strongly reduced. To support a cell-autonomous function of JunB in osteoclasts, junB was inactivated specifically in the macrophage-osteoclast lineage. Mutant mice develop an osteopetrosis-like phenotype with increased bone mass and reduced numbers of osteoclasts. Thus, these data reveal a novel function of JunB as a positive regulator controlling primarily osteoblast as well as osteoclast activity.
由于JunB是胎盘形成的必需基因,因此在胚胎本体中对其进行了条件性敲除。JunBΔ/Δ小鼠能够存活出生,但在患慢性髓性白血病样疾病之前,会因明显的细胞自主性成骨细胞和破骨细胞缺陷而发展为严重的低转换型骨质减少症。尽管据报道JunB是细胞增殖的负调节因子,但JunBΔ/Δ破骨细胞前体和成骨细胞在体内和体外均表现出增殖减少以及分化缺陷。突变的成骨细胞p16(INK4a)水平升高,但细胞周期蛋白D1和细胞周期蛋白A的表达降低。在体外成骨细胞分化过程中,Runx2会短暂增加,而诸如骨钙素和骨唾液蛋白等成熟成骨细胞标志物则会显著减少。为了支持JunB在破骨细胞中的细胞自主功能,在巨噬细胞-破骨细胞谱系中特异性地使JunB失活。突变小鼠表现出类似骨质石化的表型,骨量增加且破骨细胞数量减少。因此,这些数据揭示了JunB作为主要控制成骨细胞以及破骨细胞活性的正调节因子的新功能。