Floege Jürgen, Eitner Frank, Alpers Charles E
Department of Nephrology and Clinical Immunology, University Hospital Aachen, Pauwelsstrasse 30, D-52057 Aachen, Germany.
J Am Soc Nephrol. 2008 Jan;19(1):12-23. doi: 10.1681/ASN.2007050532. Epub 2007 Dec 12.
The PDGF system, comprising four isoforms (PDGF-A, -B, -C, and -D) and two receptor chains (PDGFR-alpha and -beta), plays important roles in wound healing, atherosclerosis, fibrosis, and malignancy. Components of the system are expressed constitutively or inducibly in most renal cells. They regulate a multitude of pathophysiologic events, ranging from cell proliferation and migration to extracellular matrix accumulation, production of pro- and anti-inflammatory mediators, tissue permeability, and regulation of hemodynamics. Genetic deletion of PDGF-B or PDGFR-beta results in an absent glomerular mesangium, whereas PDGF-C and PDGFR-alpha contribute to the formation of the renal cortical interstitium. Almost all experimental and human renal diseases are characterized by altered expression of components of the PDGF system. Infusion or systemic overexpression of PDGF-B or -D induces prominent mesangioproliferative changes and renal fibrosis. Intervention studies identified PDGF-C as a mediator of renal interstitial fibrosis and PDGF-B and -D as key factors involved in mesangioproliferative disease and renal interstitial fibrosis. These data establish PDGF as one of the best characterized growth factors in renal disease and the most potent stimulus of mesangial cell proliferation currently identified. Accordingly, targeted intervention against the various PDGF isoforms offers a promising novel therapeutic approach to renal disease.
血小板衍生生长因子(PDGF)系统由四种异构体(PDGF - A、- B、- C和 - D)和两条受体链(PDGFR - α和 - β)组成,在伤口愈合、动脉粥样硬化、纤维化和恶性肿瘤中发挥重要作用。该系统的成分在大多数肾细胞中组成性或诱导性表达。它们调节多种病理生理事件,从细胞增殖和迁移到细胞外基质积累、促炎和抗炎介质的产生、组织通透性以及血流动力学调节。PDGF - B或PDGFR - β的基因缺失导致肾小球系膜缺如,而PDGF - C和PDGFR - α则有助于肾皮质间质的形成。几乎所有的实验性和人类肾脏疾病都以PDGF系统成分的表达改变为特征。PDGF - B或 - D的输注或全身过表达会引起明显的系膜增生性改变和肾纤维化。干预研究确定PDGF - C是肾间质纤维化的介质,PDGF - B和 - D是系膜增生性疾病和肾间质纤维化的关键因素。这些数据确立了PDGF是肾脏疾病中特征最明确的生长因子之一,也是目前已确定的系膜细胞增殖的最有力刺激因素。因此,针对各种PDGF异构体的靶向干预为肾脏疾病提供了一种有前景的新型治疗方法。