Citations to this article
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.
Authors
John A. Balint, Emilios C. Kyriakides
Citations to this article (4)
| Title and authors | Publication | Year |
|---|---|---|
|
Biomarkers for disease progression and AAV therapeutic efficacy in feline Sandhoff disease
AM Bradbury, HL Gray-Edwards, JL Shirley, VJ McCurdy, AN Colaco, AN Randle, PW Christopherson, AC Bird, AK Johnson, DU Wilson, JA Hudson, NL de Pompa, DC Sorjonen, BL Brunson, M Jeyakumar, FM Platt, HJ Baker, NR Cox, M Sena-Esteves, DR Martin |
Experimental Neurology | 2014 |
|
Current Trends in Sphingolipidoses and Allied Disorders
BW Volk, L Schneck |
1976 | |
|
The amino acid composition of gray matter ganglioside-peptides of normal human and tay-sachs brains
J Palo, A Saifer |
Clinica Chimica Acta | 1970 |
|
Metabolic Conjugation and Metabolic Hydrolysis
JR Pasqualini |
Metabolic Conjugation and Metabolic Hydrolysis | 1970 |
Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)