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Research Article Free access | 10.1172/JCI105876

Studies of red cell stromal proteins in Tay-Sachs disease

John A. Balint and Emilios C. Kyriakides

1Department of Medicine (Gastroenterology), Albany Medical College, Albany, New York 12208

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1Department of Medicine (Gastroenterology), Albany Medical College, Albany, New York 12208

Find articles by Kyriakides, E. in: JCI | PubMed | Google Scholar

Published August 1, 1968 - More info

Published in Volume 47, Issue 8 on August 1, 1968
J Clin Invest. 1968;47(8):1858–1864. https://doi.org/10.1172/JCI105876.
© 1968 The American Society for Clinical Investigation
Published August 1, 1968 - Version history
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Abstract

Hemoglobin-free red blood cell ghosts of nine patients with Tay-Sachs disease and 14 normal control subjects have been analyzed for content of total protein, hexosamines, individual amino acids, and sialic acid. Red cell ghosts from Tay-Sachs' children have been shown to contain significantly increased amounts of protein, hexosamine, threonine, and serine, and probably sialic acid, each of which was increased by approximately 25% over control values. These observations suggest that the red cell membrane in patients with Tay-Sachs disease contains a significant excess of a glycoprotein or proteins, as compared with normal, and that the metabolic defect in this disease, therefore, affects glycoproteins as well as complex lipids.

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