Related Experiment Videos
Polycystin expression is temporally and spatially regulated during renal development
J Van Adelsberg1, S Chamberlain, V D'Agati
1Department of Medicine, Columbia University, College of Physicians and Surgeons, New York, New York 10032, USA.
The American Journal of Physiology
|May 1, 1997
Summary
Polycystin, the protein linked to autosomal dominant polycystic kidney disease (ADPKD), is expressed during kidney development. Its localization changes from cell membranes in early development to intracellular in mature kidneys and polycystic kidneys.
Area of Science:
- Nephrology
- Molecular Biology
- Genetics
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder.
- Mutations in the PKD1 gene are a primary cause of ADPKD.
- The PKD1 gene encodes a protein, polycystin, with predicted cell-adhesive and membrane-spanning domains.
Purpose of the Study:
- To investigate if polycystin functions as a cell adhesion molecule.
- To characterize the expression pattern of polycystin during renal development.
Main Methods:
- Antibodies were generated against specific peptides of the polycystin protein.
- Western blot analysis and immunofluorescence were used to detect polycystin in human kidney homogenates.
- Immunohistochemistry was employed to determine polycystin localization in fetal, adult, and polycystic kidneys.
Main Results:
- Antibodies recognized 485 and 245 kDa polypeptides associated with cell membranes in human fetal kidney.
- Polycystin expression was higher in fetal kidneys compared to adult kidneys.
- In fetal kidneys, polycystin localized to plasma membranes of early nephric structures but became predominantly intracellular in more mature tubules, adult kidneys, and polycystic kidneys.
Conclusions:
- The temporal and spatial expression patterns suggest polycystin's role in nephrogenesis.
- Further research is warranted to elucidate polycystin's precise function in kidney development and disease.