Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney

Christopher J. Ward, Helen Turley, Albert C.M. Ong, Margaret Comley, Simon Biddolph, Rungen Chetty, Peter J. Ratcliffe, Kevin Gatter, Peter C. Harris

Research output: Contribution to journalArticle

201 Scopus citations

Abstract

Polycystic kidney disease 1 (PKD1) is the major locus of the common genetic disorder autosomal dominant polycystic kidney disease. We have studied PKD1 mRNA, with an RNase protection assay, and found widespread expression in adult tissue, with high levels in brain and moderate signal in kidney. Expression of the PKD1 protein, polycystin, was assessed in kidney using monoclonal antibodies to a recombinant protein containing the C terminus of the molecule. In fetal and adult kidney, staining is restricted to epithelial cells. Expression in the developing nephron is most prominent in mature tubules, with lesser staining in Bowman's capsule and the proximal ureteric bud. In the nephrogenic zone, detectable signal was observed in comma- and S-shaped bodies as well as the distal branches of the ureteric bud. By contrast, uninduced mesenchyme and glomerular tufts showed no staining. In later fetal (>20 weeks) and adult kidney, strung staining persists in cortical tubules with moderate staining detected in the loops of Henle and collecting ducts. These results suggest that polycystin's major role is in the maintenance of renal epithelial differentiation and organization from early fetal life. Interestingly, polycystin expression, monitored at the mRNA level and by immunohistochemistry, appears higher in cystic epithelia, indicating that the disease does nut result from complete loss of the protein.

Original languageEnglish (US)
Pages (from-to)1524-1528
Number of pages5
JournalProceedings of the National Academy of Sciences of the United States of America
Volume93
Issue number4
DOIs
StatePublished - Feb 20 1996

Keywords

  • antibody
  • autosomal dominant polycystic kidney disease

ASJC Scopus subject areas

  • General

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