Renal cell culture using autopsy material from children with cystinosis.
Renal cell cultures were initiated using fresh autopsy material from two individuals with cystinosis, ages 5 and 8 yr. Cells obtained from collagenase treated autopsy material were grown in a selective kidney medium containing Coon's modified F12, 2.5% fetal bovine serum, transferrin, insulin, selenium, hydrocortisone, PGE1, and fibronectin. These cells had an epithelial appearance, formed domes, and were periodic acid-Schiff positive. Both tight junctions and microvilli were seen by electron microscopy. Fibroblasts had a cloning efficiency of zero in the selective medium and grew poorly compared to their growth in Coon's F12 with 10% fetal bovine serum. The lysosomal cystine content of the renal cells was greatly elevated and comparable to that of fibroblasts from cystinotic patients. Renal cell lysosomal cystine levels were only partially reduced by exposure to either pantethine or the aminothiol, cysteamine. However, exposure to either compound effectively depleted cystinotic cultured fibroblasts of their lysosomal cystine. Study of cultured renal material may have practical significance in pharmacologic considerations.
D001344:Autopsy; D002478:Cells, Cultured; D002675:Child, Preschool; D003543:Cysteamine / Q000494:pharmacology; D003553:Cystine / Q000032:analysis; D003554:Cystinosis / Q000473:pathology*; D005347:Fibroblasts / Q000187:drug effects / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000187:drug effects / Q000473:pathology*; D008297:Male; D010204:Pantetheine / Q000031:analogs & derivatives / Q000494:pharmacology
D003553:Cystine; D010204:Pantetheine; D003543:Cysteamine; C005425:pantethine