|
|
 Previous Article | Table of Contents | Next Article 
Defective glycosylphosphatidylinositol anchor synthesis in paroxysmal
nocturnal hemoglobinuria granulocytes
JF Mahoney, M Urakaze, S Hall, R DeGasperi, HM Chang, E Sugiyama, CD Warren, M Borowitz, A Nicholson-Weller and WF Rosse
Department of Medicine, Duke University, Durham, NC.
To investigate the biosynthesis of the glycosylphosphatidylinositol (GPI)
anchor in the granulocytes of paroxysmal nocturnal hemoglobinuria (PNH),
the glycolipids of granulocytes from PNH patients and normal volunteers
were biosynthetically labeled with [3H]mannose in the presence of
tunicamycin. Extracted glycolipids were examined by thin- layer
chromatography and compared with known biosynthetic intermediates. Normal
granulocytes consistently showed [3H]mannose incorporation into the
complete GPI core, several GPI biosynthetic intermediates, and dolichol
phosphate mannose (DPM). The granulocytes of 10 patients with PNH that had
no expression of CD55 and CD59 on greater than 95% of the cells were able
to incorporate [3H]mannose into DPM, but were not able to incorporate
detectable amounts into the complete GPI core. THus, PNH granulocytes do
not synthesize detectable amounts of the complete GPI core and this defect
likely accounts for the absence of GPI-linked membrane proteins on
hematopoietic cells in this syndrome.
Volume 79,
Issue 6,
pp. 1400-1403,
03/15/1992
Copyright © 1992 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. M. Risitano, R. Notaro, L. Marando, B. Serio, D. Ranaldi, E. Seneca, P. Ricci, F. Alfinito, A. Camera, G. Gianfaldoni, et al.
Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab
Blood,
April 23, 2009;
113(17):
4094 - 4100.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Wang, T. Chuhjo, S. Yasue, M. Omine, and S. Nakao
Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome
Blood,
December 1, 2002;
100(12):
3897 - 3902.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
W. H. Raskind, K. K. Niakan, J. Wolff, M. Matsushita, T. Vaughan, G. Stamatoyannopoulos, C. Watanabe, J. Rios, and H. D. Ochs
Mapping of a syndrome of X-linked thrombocytopenia with thalassemia to band Xp11-12: further evidence of genetic heterogeneity of X-linked thrombocytopenia
Blood,
April 1, 2000;
95(7):
2262 - 2268.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. E. Ware, J.-i. Nishimura, M. A. Moody, C. Smith, W. F. Rosse, and T. A. Howard
The PIG-A Mutation and Absence of Glycosylphosphatidylinositol-Linked Proteins Do Not Confer Resistance to Apoptosis in Paroxysmal Nocturnal Hemoglobinuria
Blood,
October 1, 1998;
92(7):
2541 - 2550.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Horikawa, H. Nakakuma, T. Kawaguchi, N. Iwamoto, S. Nagakura, T. Kagimoto, and K. Takatsuki
Apoptosis Resistance of Blood Cells From Patients With Paroxysmal Nocturnal Hemoglobinuria, Aplastic Anemia, and Myelodysplastic Syndrome
Blood,
October 1, 1997;
90(7):
2716 - 2722.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Miyata, N. Yamada, Y. Iida, J. Nishimura, J. Takeda, T. Kitani, and T. Kinoshita
Abnormalities of PIG-A Transcripts in Granulocytes from Patients with Paroxysmal Nocturnal Hemoglobinuria
N. Engl. J. Med.,
January 27, 1994;
330(4):
249 - 255.
[Abstract]
[Full Text]
|
|
|
|
|
