Laboratory of Cellular Oncology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Blood. 2010 Aug 12;116(6):979-87. doi: 10.1182/blood-2009-08-238360. Epub 2010 May 10.
Inactivation of p15INK4b, an inhibitor of cyclin-dependent kinases, through DNA methylation is one of the most common epigenetic abnormalities in myeloid leukemia. Although this suggests a key role for this protein in myeloid disease suppression, experimental evidence to support this has not been reported. To address whether this event is critical for premalignant myeloid disorders and leukemia development, mice were generated that have loss of p15Ink4b specifically in myeloid cells. The p15Ink4b(fl/fl)-LysMcre mice develop nonreactive monocytosis in the peripheral blood accompanied by increased numbers of myeloid and monocytic cells in the bone marrow resembling the myeloproliferative form of chronic myelomonocytic leukemia. Spontaneous progression from chronic disease to acute leukemia was not observed. Nevertheless, MOL4070LTR retrovirus integrations provided cooperative genetic mutations resulting in a high frequency of myeloid leukemia in knockout mice. Two common retrovirus insertion sites near c-myb and Sox4 genes were identified, and their transcript up-regulated in leukemia, suggesting a collaborative role of their protein products with p15Ink4b-deficiency in promoting malignant disease. This new animal model demonstrates experimentally that p15Ink4b is a tumor suppressor for myeloid leukemia, and its loss may play an active role in the establishment of preleukemic conditions.
p15INK4b 的失活,一种细胞周期蛋白依赖性激酶的抑制剂,通过 DNA 甲基化是髓系白血病中最常见的表观遗传异常之一。尽管这表明该蛋白在髓系疾病抑制中具有关键作用,但尚未有实验证据支持这一点。为了确定这种事件是否对恶性前期髓系疾病和白血病的发展至关重要,生成了 p15Ink4b 特异性缺失的髓系细胞的小鼠。p15Ink4b(fl/fl)-LysMcre 小鼠在外周血中发展为非反应性单核细胞增多症,同时骨髓中髓系和单核细胞数量增加,类似于慢性髓单核细胞白血病的髓系增生形式。未观察到从慢性疾病自发进展为急性白血病。然而,MOL4070LTR 逆转录病毒整合提供了协同的遗传突变,导致敲除小鼠中髓系白血病的高频发生。在白血病中鉴定到两个常见的逆转录病毒插入位点,靠近 c-myb 和 Sox4 基因,它们的转录物上调,提示其蛋白产物与 p15Ink4b 缺失在促进恶性疾病方面具有协作作用。这个新的动物模型实验证明,p15Ink4b 是髓系白血病的肿瘤抑制剂,其缺失可能在白血病前期条件的建立中发挥积极作用。