Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Science 28 November 1969:
Vol. 166. no. 3909, pp. 1152 - 1154
DOI: 10.1126/science.166.3909.1152
Prev | Table of Contents | Next

Articles

Cystine: Compartmentalization within Lysosomes in Cystinotic Leukocytes

J. D. Schulman 1, K. H. Bradley 1, and J. E. Seegmiller 1

1 Section on Human Biochemical Genetics, National Institute of Arthritis and Metabolic Diseases, Bethesda, Maryland 20014

The large amount of cystine compartmentalized in cystinotic leukocytes cosediments in isopycnic sucrose density gradients with dense lysosomal particles, within which it is presumably contained. Such cystine appears to be primarily noncrystalline in these organelles.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Renal metabolism of amino acids: its role in interorgan amino acid exchange.
M. C. van de Poll, P. B Soeters, N. E. Deutz, K. C. Fearon, and C. H. Dejong (2004)
Am. J. Clinical Nutrition 79, 185-197
   Abstract »    Full Text »    PDF »
Cystinosis: Antibodies and Healthy Bodies.
R. Kleta and W. A. Gahl (2002)
J. Am. Soc. Nephrol. 13, 2189-2191
   Full Text »    PDF »
Cystinosis.
W. A. Gahl, J. G. Thoene, and J. A. Schneider (2002)
N. Engl. J. Med. 347, 111-121
   Full Text »    PDF »
Cystinosis: Progress in a Prototypic Disease.
W. A. Gahl, J. G. Thoene, J. A. Schneider, S. O'Regan, M. I. Kaiser-Kupfer, and T. Kuwabara (1988)
Ann Intern Med 109, 557-569
   Abstract »    PDF »
Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis.
W. Gahl, N Bashan, F Tietze, I Bernardini, and J. Schulman (1982)
Science 217, 1263-1265
   Abstract »    PDF »
Fossilized Viscera in Primitive Echinoderms.
B. N. Haugh, B. N. Haugh, and B. M. Bell (1980)
Science 209, 653-657
   Abstract »    PDF »
Genetic Disorders of Glutathione and Sulfur Amino-Acid Metabolism: New Biochemical Insights and Therapeutic Approaches.
J. D. SCHULMAN, S. H. MUDD, J. A. SCHNEIDER, S. P. SPIELBERG, L. BOXER, J. OLIVER, L. CORASH, and M. SHEETZ (1980)
Ann Intern Med 93, 330-346
   Abstract »    PDF »
Decrease in Free Cystine Content of Cultured Cystinotic Fibroblasts by Ascorbic Acid.
W. A. Kroll and J. A. Schneider (1974)
Science 186, 1040-1042
   Abstract »    PDF »
Cystinosis: Selective Induction of Vacuolation in Fibroblasts by L-Cysteine-D-Penicillamine Disulfide.
J. D. Schulman and K. H. Bradley (1970)
Science 169, 595-597
   Abstract »    PDF »
Human Cystinosis: Intracellular Deposition of Cystine.
K. Hummeler, B. A. Zajac, M. Genel, P. G. Holtzapple, and S. Segal (1970)
Science 168, 859-860
   Abstract »    PDF »
Intracellular Cystine Content of Leukocyte Populations in Cystinosis.
J. D. Schulman, V. G. Wong, T. Kuwabara, K. H. Bradley, and J. E. Seegmiller (1970)
Arch Intern Med 125, 660-664
   Abstract »    PDF »


To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)