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

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 r...

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Hauptverfasser: Gauer, Stefan (VerfasserIn) , Kränzlin, Bettina (VerfasserIn) , Hoffmann, Sigrid (VerfasserIn) , Gretz, Norbert (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: October 23, 2017
In: Journal of histochemistry & cytochemistry
Year: 2017, Jahrgang: 65, Heft: 12, Pages: 743-755
ISSN:1551-5044
DOI:10.1369/0022155417735513
Online-Zugang:Verlag, Volltext: http://dx.doi.org/10.1369/0022155417735513
Verlag, Volltext: https://doi.org/10.1369/0022155417735513
Volltext
Verfasserangaben:Stefan Gauer, Yvonne Holzmann, Bettina Kränzlin, Sigrid C. Hoffmann, Norbert Gretz, Ingeborg A. Hauser, Margarete Goppelt-Struebe, Helmut Geiger, and Nicholas Obermüller
Beschreibung
Zusammenfassung: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.
Beschreibung:Gesehen am 17.05.2018
Beschreibung:Online Resource
ISSN:1551-5044
DOI:10.1369/0022155417735513

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