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April 1970

Intracellular Cystine Content of Leukocyte Populations in Cystinosis

Author Affiliations

Bethesda, Md; Boston; Bethesda, Md

From the Section on Human Biochemical Genetics, National Institute of Arthritis and Metabolic Diseases and National Institute of Neurological Diseases and Stroke, Bethesda, Md (Drs. Schulman, Wong, Bradley, and Seegmiller) and Howe Laboratory of Ophthalmology, Harvard Medical School, Boston (Dr. Kuwabara). Dr. Seegmiller is now with the Department of Medicine, University of California, San Diego, La Jolla.

Arch Intern Med. 1970;125(4):660-664. doi:10.1001/archinte.1970.00310040084009

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.