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Inherited Complete Deficiency of 20-Kilodalton Homologous Restriction Factor (CD59) as a Cause of Paroxysmal Nocturnal Hemoglobinuria
Open accessAbstract
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 . . .
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Citations
Cited by
Rother, R. P., Rollins, S. A., Mojcik, C. F., Brodsky, R. A., & Bell, L. (2007) · Nature Biotechnology · doi:10.1038/nbt1344 · Cited by 778
Paroxysmal nocturnal hemoglobinuria
Brodsky, R. A. (2014) · Blood · doi:10.1182/blood-2014-02-522128 · Cited by 648
Pegcetacoplan versus eculizumab in paroxysmal nocturnal hemoglobinuria
Hillmen, P., Szer, J., Weitz, I., Röth, A., Höchsmann, B., Panse, J., Usuki, K., Griffin, M., Kiladjian, J.-J., de Castro, C., et al. (2021) · New England Journal of Medicine · doi:10.1056/nejmoa2029073 · Cited by 440
Brodsky, R. A., Mukhina, G. L., Li, S., Nelson, K. L., Chiurazzi, P. L., Buckley, J. T., & Borowitz, M. J. (2000) · American Journal of Clinical Pathology · doi:10.1093/ajcp/114.3.459 · Cited by 271
Membrane proteins that protect against complement lysis
Morgan, B. P., & Meri, S. (1994) · Springer Seminars in Immunopathology · doi:10.1007/bf01837366 · Cited by 263
Metadata
- Authors
- 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.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1990-10-25
- Volume
- 323
- Issue
- 17
- Pages
- 1184-1189
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2323202050
- Cited by
- 351
- References
- 0
- FWCI
- 6.05
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Inherited Complete Deficiency of 20-Kilodalton Homologous Restriction Factor (CD59) as a Cause of Paroxysmal Nocturnal Hemoglobinuria. https://app.cochat.ai/papers/10.1056/nejm199010253231707
- The paper
- 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. (1990). Inherited Complete Deficiency of 20-Kilodalton Homologous Restriction Factor (CD59) as a Cause of Paroxysmal Nocturnal Hemoglobinuria. New England Journal of Medicine. https://doi.org/10.1056/nejm199010253231707
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
