Levéen P, Pekny M, Gebre-Medhin S, Swolin B, Larsson E, Betsholtz C
Department of Medical Biochemistry, University of Göteborg, Sweden.
Genes Dev. 1994 Aug 15;8(16):1875-87. doi: 10.1101/gad.8.16.1875.
Platelet-derived growth factor (PDGF) affects the growth, migration, and function in vitro of mesenchymal cells, but little is known about its normal physiological functions in vivo. We show here that mice deficient for PDGF B die perinatally and display several anatomical and histological abnormalities. Kidney glomerular tufts do not form, apparently because of absence of mesangial cells. Instead, a single or a few distended capillary loops fill the glomerular space. The heart and some large arteries dilate in late-stage embryos. Most PDGF B mutant embryos develop fatal hemorrhages just prior to birth. Their hematological status includes erythroblastosis, macrocytic anemia, and thrombocytopenia. On the basis of these findings, we conclude that PDGF B has crucial roles in vivo in establishing certain renal and circulatory functions.
血小板衍生生长因子(PDGF)影响间充质细胞的生长、迁移及体外功能,但对其在体内的正常生理功能却知之甚少。我们在此表明,缺乏PDGF B的小鼠在围产期死亡,并表现出多种解剖学和组织学异常。肾小球簇未形成,显然是因为缺乏系膜细胞。取而代之的是,单个或少数扩张的毛细血管袢填充了肾小球空间。在胚胎后期,心脏和一些大动脉会扩张。大多数PDGF B突变胚胎在出生前会出现致命性出血。它们的血液学状况包括成红细胞增多症、大细胞性贫血和血小板减少症。基于这些发现,我们得出结论,PDGF B在体内对建立某些肾脏和循环功能起着关键作用。