Pathophysiology and treatment of focal segmental glomerulosclerosis: the role of animal models

BMC Nephrology

Frank JMF Dor³  Ron W de Bruin¹  Martin J Hoogduijn²  Sylvana ML de Mik¹ 

[1] Laboratory of experimental surgery, Department of Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands;Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;Department of surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands

关键词: α-actinin 4;    Mpv-17;    hiv;    Puromycin aminonucleoside-induced nephrosis;    Adriamycin;    Remnant kidney;    Animal model;    Focal segmental glomerulosclerosis;   

Others  :  1082965
DOI  :  10.1186/1471-2369-14-74

 received in 2012-07-30, accepted in 2013-03-20,  发布年份 2013

【 摘 要 】

Focal segmental glomerulosclerosis (FSGS) is a kidney disease with progressive glomerular scarring and a clinical presentation of nephrotic syndrome. FSGS is a common primary glomerular disorder that causes renal dysfunction which progresses slowly over time to end-stage renal disease. Most cases of FSGS are idiopathic Although kidney transplantation is a potentially curative treatment, 40% of patients have recurrence of FSGS after transplantation. In this review a brief summary of the pathogenesis causing FSGS in humans is given, and a variety of animal models used to study FSGS is discussed. These animal models include the reduction of renal mass by resecting 5/6 of the kidney, reduction of renal mass due to systemic diseases such as hypertension, hyperlipidemia or SLE, drug-induced FSGS using adriamycin, puromycin or streptozotocin, virus-induced FSGS, genetically-induced FSGS such as via Mpv-17 inactivation and α-actinin 4 and podocin knockouts, and a model for circulating permeability factors. In addition, an animal model that spontaneously develops FSGS is discussed. To date, there is no exact understanding of the pathogenesis of idiopathic FSGS, and there is no definite curative treatment. One requirement facilitating FSGS research is an animal model that resembles human FSGS. Most animal models induce secondary forms of FSGS in an acute manner. The ideal animal model for primary FSGS, however, should mimic the human primary form in that it develops spontaneously and has a slow chronic progression. Such models are currently not available. We conclude that there is a need for a better animal model to investigate the pathogenesis and potential treatment options of FSGS.

【 授权许可】

   
2013 de Mik et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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