Soriano P
Program in Molecular Medicine, Fred Hutchinson Cancer Research Center, Seattle, Washington 98104.
Genes Dev. 1994 Aug 15;8(16):1888-96. doi: 10.1101/gad.8.16.1888.
Platelet-derived growth factor, a major mitogen and chemoattractant for a number of cell types, is implicated in the processes of wound healing, tumorigenesis, and differentiation and is recognized by two receptors, alpha and beta. To begin understanding the role of these receptors in development, beta-receptor-deficient mice were generated by gene targeting in ES cells. Mutant mice are hemorrhagic, thrombocytopenic, and severely anemic, exhibit a defect in kidney glomeruli because of a lack of mesangial cells, and die at or shortly before birth. However, many cell types and tissues that express the receptor, including major blood vessels and the heart, appear normal in the absence of the receptor. These results indicate that whereas the beta receptor is essential in certain cell types during embryonic development, its broader role may be masked because of compensation by the alpha-subunit.
血小板衍生生长因子是多种细胞类型的主要促有丝分裂剂和趋化因子，参与伤口愈合、肿瘤发生和分化过程，并可被α和β两种受体识别。为了开始了解这些受体在发育中的作用，通过对胚胎干细胞进行基因靶向操作，培育出了β受体缺陷型小鼠。突变小鼠有出血、血小板减少和严重贫血症状，由于缺乏系膜细胞，肾小球存在缺陷，在出生时或出生前不久死亡。然而，许多表达该受体的细胞类型和组织，包括主要血管和心脏，在缺乏该受体的情况下看起来正常。这些结果表明，虽然β受体在胚胎发育过程中对某些细胞类型至关重要，但其更广泛的作用可能由于α亚基的补偿作用而被掩盖。
