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Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS).

Publication ,  Journal Article
Joseph, KC; Gockerman, JP; Alving, CR
Published in: J Lab Clin Med
January 1975

The lipids derived from the erythrocytes of two siblings with clinical congenital dyserythropietic anemia Type II (CDA-II) and one sibling without clinical evidence of CDA-II underwent a detailed analysis. The total phospholipids, total cholesterol, and cholesterol: phosphate ratio were normal in all siblings. In the two clinically affected siblings there was a significant increase in the phosphatidyl choline with a concomitant decrease in the other phospholipids. The fatty acids of phosphatidyl choline from the CDA-II erythrocytes were normal. The total gylcosphingolipids were increased in all siblings. The glycosphingolipids, di- , tri-, and tetrahexosyl ceramides were increased 1.5- to 10-fold over controls in the clinically affected siblings. The increases were present but less striking in the clinically unaffected sibling. Gluococerebroside was not significantly increased in any of the siblings. The possible causes and significance of the lipid changes are discussed.

Duke Scholars

Published In

J Lab Clin Med

ISSN

0022-2143

Publication Date

January 1975

Volume

85

Issue

1

Start / End Page

34 / 40

Location

United States

Related Subject Headings

  • Sphingolipids
  • Phospholipids
  • Phosphatidylcholines
  • Lipids
  • Humans
  • General Clinical Medicine
  • Erythropoiesis
  • Erythrocytes
  • Chromatography, Thin Layer
  • Cholesterol
 

Citation

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MLA
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Joseph, K. C., Gockerman, J. P., & Alving, C. R. (1975). Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS). J Lab Clin Med, 85(1), 34–40.
Joseph, K. C., J. P. Gockerman, and C. R. Alving. “Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS).J Lab Clin Med 85, no. 1 (January 1975): 34–40.
Joseph KC, Gockerman JP, Alving CR. Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS). J Lab Clin Med. 1975 Jan;85(1):34–40.
Joseph, K. C., et al. “Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS).J Lab Clin Med, vol. 85, no. 1, Jan. 1975, pp. 34–40.
Joseph KC, Gockerman JP, Alving CR. Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS). J Lab Clin Med. 1975 Jan;85(1):34–40.
Journal cover image

Published In

J Lab Clin Med

ISSN

0022-2143

Publication Date

January 1975

Volume

85

Issue

1

Start / End Page

34 / 40

Location

United States

Related Subject Headings

  • Sphingolipids
  • Phospholipids
  • Phosphatidylcholines
  • Lipids
  • Humans
  • General Clinical Medicine
  • Erythropoiesis
  • Erythrocytes
  • Chromatography, Thin Layer
  • Cholesterol