TY - JOUR
T1 - Deficient biosynthesis of N-acetylglucosaminyl-phosphatidylinositol, the first intermediate of glycosyl phosphatidylinositol anchor biosynthesis, in cell lines established from patients with paroxysmal nocturnal hemoglobinuria
AU - Takahashi, Minoru
AU - Takeda, Junji
AU - Hirose, Shinichi
AU - Hyman, Robert
AU - Inoue, Norimitsu
AU - Miyata, Toshio
AU - Ueda, Etsuko
AU - Kitani, Teruo
AU - Edward Medof, M.
AU - Kinoshita, Taroh
N1 - Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 1993/2/1
Y1 - 1993/2/1
N2 - Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disorder caused by a deficiency of biosynthesis of the glycosyl phosphatidylinositol (GPI) anchor, but the biochemical defect is not completely understood. In the present study, we have analyzed affected cell lines established recently from two Japanese patients with PNH. Two lines of evidence indicate that these cells do not synthesize N-acetylglucosaminyl-phosphatidylinositol, the first intermediate in the GPI anchor biosynthesis. First, somatic cell hybridization analysis using Thy-l-deficient murine thymoma cell lines with known biochemical defects as fusion partners showed that the PNH cell lines belong to complementation class A, which is known not to synthesize N-acetylglucosaminylphosphatidylinositol. Second, analysis of in vitro glycolipid biosynthesis demonstrated that cell lysates of these PNH cell lines in fact did not support biosynthesis of N-acetylglucosaminylphosphatidylinositol. Thus, we have characterized for the first time the exact biochemical defect leading to PNH.
AB - Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disorder caused by a deficiency of biosynthesis of the glycosyl phosphatidylinositol (GPI) anchor, but the biochemical defect is not completely understood. In the present study, we have analyzed affected cell lines established recently from two Japanese patients with PNH. Two lines of evidence indicate that these cells do not synthesize N-acetylglucosaminyl-phosphatidylinositol, the first intermediate in the GPI anchor biosynthesis. First, somatic cell hybridization analysis using Thy-l-deficient murine thymoma cell lines with known biochemical defects as fusion partners showed that the PNH cell lines belong to complementation class A, which is known not to synthesize N-acetylglucosaminylphosphatidylinositol. Second, analysis of in vitro glycolipid biosynthesis demonstrated that cell lysates of these PNH cell lines in fact did not support biosynthesis of N-acetylglucosaminylphosphatidylinositol. Thus, we have characterized for the first time the exact biochemical defect leading to PNH.
UR - http://www.scopus.com/inward/record.url?scp=0027412005&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0027412005&partnerID=8YFLogxK
U2 - 10.1084/jem.177.2.517
DO - 10.1084/jem.177.2.517
M3 - Article
C2 - 8426120
AN - SCOPUS:0027412005
VL - 177
SP - 517
EP - 521
JO - Journal of Experimental Medicine
JF - Journal of Experimental Medicine
SN - 0022-1007
IS - 2
ER -