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Genomic organization of the. alpha. subunit of human complement C8

Conference · · FASEB Journal (Federation of American Societies for Experimental Biology); (United States)
OSTI ID:5148179
; ;  [1]
  1. Univ. of South Carolina, Columbia (United States)
Human C8 is one of five terminal complement components (C5b, C6, C7, C8, C9) that interact to form the cytolytic C5b-9 complex. It is composed of three nonidentical subunits ({alpha}, {beta}, {gamma}) which are encoded in separate genes. C8{alpha} and C8{beta} are homologous to each other and to C6, C7 and C9. All share common structural motifs that include cysteine-rich thrombospondin, LDL receptor and epidermal growth factor-like segments as well as potential membrane interacting domains. At the genomic level, only C9 has been characterized thus far and was found to contain eleven exons spanning >90 kb. Correlation between exon boundaries and the above structural motifs in C9 was found to be limited. To further define evolutionary relationships amongst the terminal components and facilitate molecular studies of hereditary C8 deficiencies, the authors isolated and characterized the human C8{alpha} gene. Results revealed that C8{alpha} is also composed of eleven exons that span > 70kb. As in C9, several large introns separate exon clusters. Furthermore, exon lengths and boundaries correlate well with those in C9, indicating a close ancestral relationship between the two genes.
OSTI ID:
5148179
Report Number(s):
CONF-9104107--
Conference Information:
Journal Name: FASEB Journal (Federation of American Societies for Experimental Biology); (United States) Journal Volume: 5:5
Country of Publication:
United States
Language:
English