ABNORMAL KIDNEY DEVELOPMENT AND HEMATOLOGICAL DISORDERS IN PDGF BETA-RECEPTOR MUTANT MICE
ABNORMAL KIDNEY DEVELOPMENT AND HEMATOLOGICAL DISORDERS IN PDGF BETA-RECEPTOR MUTANT MICE
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DOI：
10.1101/gad.8.16.1888
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发表时间：
1994-08-15
影响因子：
10.5
通讯作者：
SORIANO, P
中科院分区：
文献类型：
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作者：
SORIANO, P
血小板衍生生长因子是许多细胞类型的主要有丝分裂原和化学引诱物，与伤口愈合、肿瘤发生和分化过程有关，并被两种受体α和β识别。为了开始理解这些受体在发育中的作用，通过在ES细胞中的基因靶向产生β受体缺陷小鼠。突变小鼠是出血性的、血小板减少的和严重贫血的，由于缺乏系膜细胞而表现出肾小球缺陷，并且在出生时或出生前不久死亡。然而，许多表达受体的细胞类型和组织，包括主要血管和心脏，在缺乏受体的情况下表现正常。这些结果表明，尽管β受体在胚胎发育期间的某些细胞类型中是必不可少的，但由于α亚基的补偿，其更广泛的作用可能被掩盖。
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.