SEARCH:   Advanced

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Wang, L. Articles by Juji, T. Search for Related Content PubMed PubMed Citation Articles by Wang, L. Articles by Juji, T. Related Collections Red Cells Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Molecular Cloning and Characterization of Decay-Accelerating Factor Deficiency in Cromer Blood Group Inab Phenotype Li Wang, Makoto Uchikawa, Hatsue Tsuneyama, Katsushi Tokunaga, Kenji Tadokoro, and Takeo Juji From the Japanese Red Cross Central Blood Center, Tokyo, Japan 150; the Department of Biological Sciences, Graduate School of Science, The University of Tokyo, Tokyo, Japan; and the Department of Human Genetics, Graduate School of International Health, The University of Tokyo, Tokyo, Japan. An additional decay-accelerating factor (DAF) mutation, designated as Inab phenotype in the Cromer blood group system, was recently identified in a 28-year-old Japanese woman (H.A.). The red blood cells of H.A., like those of other Inab phenotype individuals, were negative for Cromer system antigens, Cra, Tca, Dra, UMC, and IFC. The deficiency of DAF on the red blood cells of H.A. has been shown by immunoblotting with a murine monoclonal antibody to DAF. Molecular analysis has shown that H.A. is homozygous for a single nucleotide substitution, C1579A, at the position 24 bp upstream of the 3-end of exon 2 of the DAF gene. This substitution causes the activation of a novel cryptic splice site and results in the production of mRNA with a 26 bp deletion. The deletion introduces a reading frame shift and creates a stop codon immediately downstream of the deletion. Translation of mRNA would be terminated at the first amino acid residue of the second short consensus repeat (SCR2) domain (exon 3) of DAF. The functional domains of DAF's complement regulatory activity and the carboxy-terminal signal domains for glycosylphosphatidylinositol (GPI) anchoring are predicted to be lacking in H.A. Thus, there would be no DAF present on the cell surface. Blood, Vol. 91 No. 2 (January 15), 1998: pp. 680-684 © 1998 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: T. Miwa, M. A. Maldonado, L. Zhou, K. Yamada, G. S. Gilkeson, R. A. Eisenberg, and W.-C. Song Decay-Accelerating Factor Ameliorates Systemic Autoimmune Disease in MRL/lpr Mice via Both Complement-Dependent and -Independent Mechanisms Am. J. Pathol., April 1, 2007; 170(4): 1258 - 1266. [Abstract] [Full Text] [PDF] T. Miwa, L. Zhou, B. Hilliard, H. Molina, and W.-C. Song Crry, but not CD59 and DAF, is indispensable for murine erythrocyte protection in vivo from spontaneous complement attack Blood, May 15, 2002; 99(10): 3707 - 3716. [Abstract] [Full Text] [PDF] H. Sogabe, M. Nangaku, Y. Ishibashi, T. Wada, T. Fujita, X. Sun, T. Miwa, M. P. Madaio, and W.-C. Song Increased Susceptibility of Decay-Accelerating Factor Deficient Mice to Anti-Glomerular Basement Membrane Glomerulonephritis J. Immunol., September 1, 2001; 167(5): 2791 - 2797. [Abstract] [Full Text] [PDF] X. Sun, C. D. Funk, C. Deng, A. Sahu, J. D. Lambris, and W.-C. Song Role of decay-accelerating factor in regulating complement activation on the erythrocyte surface as revealed by gene targeting PNAS, January 19, 1999; 96(2): 628 - 633. [Abstract] [Full Text] [PDF]

	Copyright © 1998 by American Society of Hematology         Online ISSN: 1528-0020