Intracellular Cystine Content of Leukocyte Populations in Cystinosis

Authors:
  • JSJoseph D. Schulman
    Joseph D. Schulman
  • VWVernon G. Wong
    Vernon G. Wong
  • TKToichiro Kuwabara
    Toichiro Kuwabara
  • KBKatherine H. Bradley
    Katherine H. Bradley
  • JSJ. Edwin Seegmiller
    J. Edwin Seegmiller
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Abstract
An increased content of free cystine has been found in phagocytic blood cells, the polymorphonuclear leukocytes and monocytes, but not in lymphocytes of patients with cystinosis. Electron micrographs showed no intracellular crystals, suggesting that cystine may be primarily in noncrystalline form in these cells. A primary derangement of cystine metabolism or transport within the lysosomes of cells carrying out active endocytosis could account for these observations.