Transport studies in primary cultures of mouse renal epithelial cells

• Main Author: Bell, Cindy Lea.
• Format: Others
• Language: en
• Published: McGill University 1986
• Subjects: Mice > Genetics.; Mice > Cytology.
• Online Access: http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=75363

The Hyp (hypophosphatemic) mouse, a murine homologue of X-linked hypophosphatemia (XLH) in man, is a Mendelian disorder of phosphate (Pi) homeostasis. The mutant genotype is characterized by abnormal Pi transport at the brush border membrane (BBM) of the proximal tubule and a defect in renal metabolism of vitamin D$ sb3$. The exact nature of these defects has not been elucidated. === In order to determine if the defect is intrinsic to the renal cell or dependent upon an extrinsic humoral factor, I established primary cultures of renal epithelial cells from normal and Hyp mouse kidney. The cultures demonstrated several differentiated properties of epithelial cells of the renal proximal tubule, the site of the Pi transport defect in the Hyp mouse. === Primary cultures initiated from Hyp mice had decreased Pi transport (expressed as an uptake ratio, Pi/$ alpha$-MG), and increased production of 24,25 dihydroxyvitamin D$ sb3$. These results provide evidence for the intrinsic nature of the primary defect in the Hyp mouse. === This appears to be the first time that expression of a mutant transport gene has been demonstrated in cultured renal cells.