Work

Inherited Complete Deficiency of 20-Kilodalton Homologous Restriction Factor (CD59) as a Cause of Paroxysmal Nocturnal Hemoglobinuria

Year: 1990

Type: article

Abstract: 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 . . .

Source: New England Journal of Medicine

DOI: 10.1056/nejm199010253231707

Authors: Manabu Yamashina, Etsuko Ueda, Taroh Kinoshita, Tsuyoshi Takami, Akitsugu Ojima, Hiromasa Ono, Hiroshi Tanaka, Naomi Kondo, Tadao Orii, Noriko Okada, Hidechika Okada, Kozo Inoue, Teruo Kitani

Institutions: Gifu University, Research Institute for Microbial Diseases, The University of Osaka, The University of Osaka, Fukuoka University, Nagoya City University

Language: English

Topic: Complement system in diseases

Subfield: Immunology

Field: Immunology and Microbiology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W2323202050

FWCI: 5.649

Cites: 44

Cited by: 385

Related to: 10