Aberrant Expression of Polycystin-1 in Renal Cell Tumors

Journal Title: Macedonian Journal of Medical Sciences (MJMS) - Year 2008, Vol 1, Issue 1


Polycystin-1 (PC1) is a cellular transmembrane protein coded by the polycystic kidney disease (PKD1) gene, prevalently expressed in developing/mature kidney and in autosomal polycystic kidney disease (ADPKD). Limited data are available concerning the PC1 involvement in renal tumorigenenesis. Polycystin-1 expression was evaluated in 8 clear cell renal cell carcinomas (RCCs), 7 tubulopapillary cell type tumors, 3 solid RCCs developed in patients with von Hippel-Lindau disease (VHLD), one RCC developed in a patient on chronic haemodialysis and one angiomyolipoma in a patient with Tuberous sclerosis (TS). In the normal kidney, consistent level of polycystin-1 was detected in distal tubules, collecting duct, glomerular podocytes and vascular smooth muscle cells. The strongest immunoreactivity against polycystin-1 was observed in epithelial cells lining the cystic components in all ADPKD tissues. Five cases of clear cell type RCCs and two-tubulopapillary cell type RCCs consistently expressed PC1. In the VHL disease associated renal carcinomas, both the neoplastic cells and cystic tissue areas weakly expressed PC1. In TS-associated angiomyolipoma, the vascular component was PC1 positive, while the tumoral cells were scarcely stained. The present report indicates consistent expression of the PKD1 gene product polycystin-1, in normal kidney, ADPKD tissues, and renal cell carcinomas. The findings suggest that the level of PC1 expression is linked to tumor cell type, being a more frequent event in clear cell RCC.

Authors and Affiliations

Jean Gogusev| INSERM U507, Hôpital Necker France, Yves Chretien| Service d’Urologie, Hôpital Necker France, Dominique Droz| INSERM U507, Hôpital NeckerService d’Anatomie Pathologique, Hopital NeckerEnfants Malades, Paris, France


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  • EP ID EP8582
  • DOI 10.3889/MJMS.1857-5773.2008.0004
  • Views 350
  • Downloads 26

How To Cite

Jean Gogusev, Yves Chretien, Dominique Droz (2008). Aberrant Expression of Polycystin-1 in Renal Cell Tumors. Macedonian Journal of Medical Sciences (MJMS), 1(1), 18-25. https://europub.co.uk/articles/-A-8582