CTGF is expressed during cystic remodeling in the PKD/Mhm (cy/+) rat model for Autosomal-Dominant Polycystic Kidney Disease (ADPKD)

• Verfasst von: Gauer, Stefan [VerfasserIn]
• Verfasst von: Kränzlin, Bettina [VerfasserIn]
• Verfasst von: Hoffmann, Sigrid [VerfasserIn]
• Verfasst von: Gretz, Norbert [VerfasserIn]
• Titel: CTGF is expressed during cystic remodeling in the PKD/Mhm (cy/+) rat model for Autosomal-Dominant Polycystic Kidney Disease (ADPKD)
• Verf.angabe: Stefan Gauer, Yvonne Holzmann, Bettina Kränzlin, Sigrid C. Hoffmann, Norbert Gretz, Ingeborg A. Hauser, Margarete Goppelt-Struebe, Helmut Geiger, and Nicholas Obermüller
• E-Jahr: 2017
• Jahr: October 23, 2017
• Umfang: 13 S.
• Fussnoten: Gesehen am 17.05.2018
• Titel Quelle: Enthalten in: Journal of histochemistry & cytochemistry
• Ort Quelle: London [u.a.] : Sage Publ., 1953
• Jahr Quelle: 2017
• Band/Heft Quelle: 65(2017), 12, Seite 743-755
• ISSN Quelle: 1551-5044
• DOI: doi:10.1369/0022155417735513
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 157527132X

Abstract

Connective tissue growth factor (CTGF, also named CCN2) plays an important role in the development of tubulointerstitial fibrosis, which most critically determines the progression to end-stage renal failure in autosomal-dominant polycystic kidney disease (ADPKD), the most common genetically caused renal disease. We determined CTGF expression in a well-characterized animal model of human ADPKD, the PKD/Mhm (cy/+) rat. Kidneys of 12 weeks old (cy/+) as well as (+/+) non-affected rats were analyzed for CTGF RNA and protein expression by RT-PCR, Northern and Western blot analyses, in situ hybridization, and IHC. Besides the established expression of CTGF in glomerular cells in kidneys of wild-type (+/+) animals, in (cy/+) rats, CTGF mRNA and protein were robustly expressed in interstitial, stellate-shaped cells, located in a scattered pattern underlying the cystic epithelium and in focal areas of advanced tubulointerstitial remodeling. Renal CTGF mRNA and protein expression levels were significantly higher in (cy/+) rats compared with their (+/+) littermates. Detection of CTGF expression in cells adjacent to cystic epithelium and in areas of marked fibrosis suggests a role in the local response to cyst development and indicates that CTGF may be a relevant factor contributing to tubulointerstitial fibrosis in polycystic kidney disease.