Program of Inflammatory Disease Research, Infectious and Inflammatory Disease Center, Cancer Center, Sanford-Burnham Medical Research Institute, La Jolla, CA 92037, USA.
J Exp Med. 2010 Oct 25;207(11):2407-20. doi: 10.1084/jem.20091962. Epub 2010 Oct 18.
The inositol phosphatases phosphatase and tensin homologue (PTEN) and Src homology 2 domain-containing inositol phosphatase (SHIP) negatively regulate phosphatidylinositol-3-kinase (PI3K)-mediated growth, survival, and proliferation of hematopoietic cells. Although deletion of PTEN in mouse T cells results in lethal T cell lymphomas, we find that animals lacking PTEN or SHIP in B cells show no evidence of malignancy. However, concomitant deletion of PTEN and SHIP (bPTEN/SHIP(-/-)) results in spontaneous and lethal mature B cell neoplasms consistent with marginal zone lymphoma or, less frequently, follicular or centroblastic lymphoma. bPTEN/SHIP(-/-) B cells exhibit enhanced survival and express more MCL1 and less Bim. These cells also express low amounts of p27(kip1) and high amounts of cyclin D3 and thus appear poised to undergo proliferative expansion. Unlike normal B cells, bPTEN/SHIP(-/-) B cells proliferate to the prosurvival factor B cell activating factor (BAFF). Interestingly, although BAFF availability may promote lymphoma progression, we demonstrate that BAFF is not required for the expansion of transferred bPTEN/SHIP(-/-) B cells. This study reveals that PTEN and SHIP act cooperatively to suppress B cell lymphoma and provides the first direct evidence that SHIP is a tumor suppressor. As such, assessment of both PTEN and SHIP function are relevant to understanding the etiology of human B cell malignancies that exhibit augmented activation of the PI3K pathway.
磷酸肌醇 3-激酶(PI3K)介导的造血细胞生长、存活和增殖受磷酸肌醇 3-磷酸酶和张力蛋白同源物(PTEN)和Src 同源 2 结构域含肌醇磷酸酶(SHIP)负调控。虽然在小鼠 T 细胞中缺失 PTEN 会导致致命性 T 细胞淋巴瘤,但我们发现缺失 B 细胞中的 PTEN 或 SHIP 的动物没有恶性肿瘤的证据。然而,PTEN 和 SHIP 的同时缺失(bPTEN/SHIP(-/-))导致自发和致命的成熟 B 细胞肿瘤,与边缘区淋巴瘤一致,或较少见,滤泡性或中心母细胞性淋巴瘤。bPTEN/SHIP(-/-)B 细胞表现出增强的存活能力,并表达更多的 MCL1 和更少的 Bim。这些细胞还表达低量的 p27(kip1)和高量的 cyclin D3,因此似乎准备进行增殖扩张。与正常 B 细胞不同,bPTEN/SHIP(-/-)B 细胞增殖到生存因子 B 细胞激活因子(BAFF)。有趣的是,尽管 BAFF 的可用性可能促进淋巴瘤的进展,但我们证明 BAFF 不是转移的 bPTEN/SHIP(-/-)B 细胞扩张所必需的。这项研究揭示了 PTEN 和 SHIP 协同作用抑制 B 细胞淋巴瘤,并提供了 SHIP 是肿瘤抑制因子的第一个直接证据。因此,评估 PTEN 和 SHIP 的功能对于理解表现出 PI3K 途径过度激活的人类 B 细胞恶性肿瘤的病因具有重要意义。