INHERITED COMPLETE DEFICIENCY OF 20-KILODALTON HOMOLOGOUS RESTRICTION FACTOR-(CD59) AS A CAUSE OF PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA

被引：277

作者：

YAMASHINA, M

UEDA, E

KINOSHITA, T

TAKAMI, T

OJIMA, A

ONO, H

TANAKA, H

KONDO, N

ORII, T

OKADA, N

OKADA, H

INOUE, K

KITANI, T

机构：

[1] GIFU UNIV,SCH MED,DEPT PEDIAT,GIFU 500,JAPAN

[2] OSAKA UNIV,MICROBIAL DIS RES INST,DEPT CLIN RES,DIV INTERNAL MED,SUITA,OSAKA 565,JAPAN

[3] OSAKA UNIV,SCH MED,DEPT BACTERIOL,SUITA,OSAKA 565,JAPAN

[4] GIFU PREFECTURAL GIFU HOSP,DEPT PEDIAT,GIFU,JAPAN

[5] FUKUOKA UNIV,SCH MED,DEPT MICROBIOL,FUKUOKA 81401,JAPAN

[6] NAGOYA CITY UNIV,SCH MED,DEPT MOLEC BIOL,NAGOYA,AICHI 467,JAPAN

来源：

NEW ENGLAND JOURNAL OF MEDICINE | 1990年 / 323卷 / 17期

关键词：

D O I：

10.1056/NEJM199010253231707

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

PAROXYSMAL nocturnal hemoglobinuria is a rare acquired disease caused by an unusual susceptibility of erythrocytes to the lytic action of complement. The abnormal erythrocytes are thought to originate from the clonal proliferation of bone marrow progenitors altered by somatic mutation.1 Erythrocytes from patients with paroxysmal nocturnal hemoglobinuria have been shown to be deficient in membrane glycoproteins, such as decay-accelerating factor (DAF)2,3 and a factor called either homologous restriction factor4 (HRF) or C8-binding protein5 (C8bp). DAF can inhibit the formation and induce the dissociation of C3 convertases of the classical and alternative pathways of complement activation.6 7 8 HRF/C8bp appears to limit the. © 1990, Massachusetts Medical Society. All rights reserved.

引用

页码：1184 / 1189

页数：6

共 44 条

[1]

Bayer E A, 1979, Methods Enzymol, V62, P308

[2]

CHOW FL, 1986, BLOOD, V67, P893

[3] CD59, AN LY-6-LIKE PROTEIN EXPRESSED IN HUMAN LYMPHOID-CELLS, REGULATES THE ACTION OF THE COMPLEMENT MEMBRANE ATTACK COMPLEX ON HOMOLOGOUS CELLS [J].