Renal cell culture using autopsy material from children with cystinosis |
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Authors: | Ocean L Pellett Margaret L Smith Smith Jess G Thoene Jerry A Schneider Adam J Jonas |
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Institution: | (1) Department of Pediatrics, University of California, San Diego, 6431 Fannin, Street, 92093 La Jolla, California;(2) Department of Pediatrics, University of Michigan, 6431 Fannin Street, 48109 Ann Arbor, Michigan;(3) Department of Pediatrics, University of Texas Medical School at Houston, 6431 Fannin Street, 77030 Houston, Texas |
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Abstract: | Summary 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.
This work was supported by Grants AM 01074-01, AM 18434, and GM 17702 from the National Institutes of Health, Bethesda, MD. |
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Keywords: | renal cells renal culture autopsy material cystinosis |
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