title: Adult, fetal, and polycystic kidney expression of polycystin, the polycystic kidney disease-1 gene product.
abstract: The polycystic kidney disease-1 gene, which is mutated in the majority of patients with autosomal dominant polycystic kidney disease, has been identified. The protein encoded by this gene, polycystin, has no homology with any gene known thus far. To gain more insight into the function of polycystin, we raised antibodies against synthetic peptides and a fusion protein corresponding to the sequence of two different fragments of polycystin. Two of the antibodies were capable of immunoprecipitating an in vitro transcription and translation product corresponding to a fragment of polycystin. In the cyst-lining epithelium of polycystic kidney disease-1 patients, a strong staining was observed. In normal adult and embryonic kidney tissues, expression was seen in the epithelium of all tubular structures and in the glomerular parietal and visceral epithelium (podocytes), although the podocytes were mainly recognized on cryosections and not on paraffin sections. A double-labeled immunofluorescence with one of the polycystin antibodies and the monoclonal antibody 8G8 ascertained that within the glomerular tuft podocytes were recognized.
authors.affiliation: Department of Human Genetics, Sylvius Laboratories, Leiden University, The Netherlands.
authors.forename: D J
authors.initials: DJ
authors.lastname: Peters
authors.forename: L
authors.initials: L
authors.lastname: Spruit
authors.forename: R
authors.initials: R
authors.lastname: Klingel
authors.forename: F
authors.initials: F
authors.lastname: Prins
authors.forename: H J
authors.initials: HJ
authors.lastname: Baelde
authors.forename: P C
authors.initials: PC
authors.lastname: Giordano
authors.forename: L F
authors.initials: LF
authors.lastname: Bernini
authors.forename: E
authors.initials: E
authors.lastname: de Heer
authors.forename: M H
authors.initials: MH
authors.lastname: Breuning
authors.forename: J A
authors.initials: JA
authors.lastname: Bruijn
mesh_terms: D000328:Adult; D000595:Amino Acid Sequence; D015153:Blotting, Western; D005314:Embryonic and Fetal Development / Q000235:genetics*; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000737:chemistry / Q000196:embryology* / Q000378:metabolism*; D008969:Molecular Sequence Data; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics / Q000378:metabolism*; D011233:Precipitin Tests; D014176:Protein Biosynthesis; D011506:Proteins / Q000737:chemistry; D050396:TRPP Cation Channels
chemical_list: D011506:Proteins; D050396:TRPP Cation Channels