Pathogenesis of renal lesion in Fechtner syndrome
- VernacularTitle:Fechtner综合征肾脏病理特点及机制探讨
- Author:
Haiyan YANG
;
Zhaoyue WANG
;
Guoyuan LU
;
Xiaojuan ZHAO
;
Zhigang ZHANG
;
Muyi GUO
;
Xia BAI
;
Changgeng RUAN
- Publication Type:Journal Article
- Keywords:
Nonmusch myosin type IIA;
Mutation;
Podocytes;
Fechtner syndrome;
Cone,MYH9
- From:
Chinese Journal of Nephrology
2008;24(5):328-331
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the pathogenesis of renal lesion in Fechtner syndrome . Methods Pathological characteristics of kidney tissues from Fechtner syndrome patients were explored by HE staining, immunochemistry, immunofluorescence and electron microscopy . Results Immunochemistry analysis showed that non-muscle myosin heavy chain IIA (NMMHC-IIA)was expressed in podocytes of giomeruli and distal convoluted tube, and was faintly expressed in the brush border of proximal tube . Histological examination demonstrated glomerulosclerosis and decreased expression of NMMHC-IIA in abnormal podocytes . Through standard immunofluorecence, the expression of NMMHC-IIA in patient's podocyte was higher than that in normal pedocytes . The fusion of foot process and microvillus were detected by electron microscopy . Conclusion Abnormal NMMHC-IIA aggregates in the glomeruli podocyts and foot process fusion accompanied with appearance of microvillus leads to renal lesion in Fechtuer syndrome .
