Dysfunctional C8 beta chain in patients with C8 deficiency

Details

Serval ID
serval:BIB_7CD72919973D
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Dysfunctional C8 beta chain in patients with C8 deficiency
Journal
Scandinavian Journal of Immunology
Author(s)
Tschopp  J., Penea  F., Schifferli  J., Spath  P.
ISSN
0300-9475 (Print)
Publication state
Published
Issued date
12/1986
Volume
24
Number
6
Pages
715-20
Notes
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Dec
Abstract
Two sera from unrelated individuals, each lacking C8 activity, were examined by Western blot analysis. Using antisera raised against whole C8, the two sera are shown to lack the C8 beta chain, indicating a C8 beta deficiency, which is frequently observed in cases of dysfunctional C8. In contrast, by means of a specific anti-C8-beta antiserum, a C8 beta-like polypeptide chain of apparently identical molecular weight compared to normal C8 beta was detected. Digestion of normal and dysfunctional C8 beta with Staphylococcus aureus V8 protease revealed distinct differences in the enzymatic digestion pattern. We conclude that the dysfunction in the C8 protein in these two patients resides in the dysfunctional C8 beta chain, and that this form of C8 deficiency is distinct from C8 deficiencies previously reported, in which one or both C8 subunits are lacking.
Keywords
Adult Child Complement C8/analysis/*deficiency/immunology Electrophoresis, Polyacrylamide Gel Female Humans Male Molecular Weight Peptide Mapping
Pubmed
Web of science
Create date
24/01/2008 15:19
Last modification date
20/08/2019 14:38
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