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Glycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step.

Publication ,  Journal Article
Norris, J; Hall, S; Ware, RE; Kamitani, T; Chang, HM; Yeh, E; Rosse, WF
Published in: Blood
February 1, 1994

The defect in the biosynthesis of the glycosyl-phosphatidyl inositol (GPI) anchor in paroxysmal nocturnal hemoglobinuria (PNH) appears to be in the initial steps. In biosynthetic studies using [3H]mannose, abnormal granulocytes of eight patients, and B lymphocytes transformed by Epstein-Barr virus of six different patients synthesized dolichyl phosphoryl mannose, but little or no later mannosylated intermediates. When fused with murine cell lines known to be deficient at different biosynthetic steps of the GPI anchor, the GPI-anchor-deficient granulocytes of 21/21 patients and lymphocytes from 6/6 patients complemented all murine cell lines except those of class A; cells of this class are not able to add N-acetylglucosamine to phosphatidylinositol. These studies indicate that the defect in GPI-anchor synthesis in PNH is early in the pathway, and is the same as that of class A mutants, but may be partial in some patients, resulting in the production of small amounts of mannosylated intermediates.

Duke Scholars

Published In

Blood

ISSN

0006-4971

Publication Date

February 1, 1994

Volume

83

Issue

3

Start / End Page

816 / 821

Location

United States

Related Subject Headings

  • Membrane Glycoproteins
  • Lymphocytes
  • Immunology
  • Humans
  • Hemoglobinuria, Paroxysmal
  • Glycosylphosphatidylinositols
  • Cell Line, Transformed
  • CD59 Antigens
  • Antigens, CD
  • 3213 Paediatrics
 

Citation

APA
Chicago
ICMJE
MLA
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Norris, J., Hall, S., Ware, R. E., Kamitani, T., Chang, H. M., Yeh, E., & Rosse, W. F. (1994). Glycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step. Blood, 83(3), 816–821.
Norris, J., S. Hall, R. E. Ware, T. Kamitani, H. M. Chang, E. Yeh, and W. F. Rosse. “Glycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step.Blood 83, no. 3 (February 1, 1994): 816–21.
Norris J, Hall S, Ware RE, Kamitani T, Chang HM, Yeh E, et al. Glycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step. Blood. 1994 Feb 1;83(3):816–21.
Norris J, Hall S, Ware RE, Kamitani T, Chang HM, Yeh E, Rosse WF. Glycosyl-phosphatidylinositol anchor synthesis in paroxysmal nocturnal hemoglobinuria: partial or complete defect in an early step. Blood. 1994 Feb 1;83(3):816–821.

Published In

Blood

ISSN

0006-4971

Publication Date

February 1, 1994

Volume

83

Issue

3

Start / End Page

816 / 821

Location

United States

Related Subject Headings

  • Membrane Glycoproteins
  • Lymphocytes
  • Immunology
  • Humans
  • Hemoglobinuria, Paroxysmal
  • Glycosylphosphatidylinositols
  • Cell Line, Transformed
  • CD59 Antigens
  • Antigens, CD
  • 3213 Paediatrics