Year: 1964
Type: article
Abstract: Thus, symptoms of hereditary angioneurotic edema were associated with enhanced generation of C'1-esterase.Ratnoff and Lepow have dem- onstrated that C'1-esterase can increase vascular permeability in the skin of the guinea pig and that purified serum inhibitor of C'1-esterase can impair this response (10).C'1-esterase may also serve to increase vascular permeability in heredi- tary angioneurotic edema.Methods Nomenclature.Complement activity resides in a group of proteins in fresh serum.Although required for im- mune hemolysis and immune cellular injury in vitro, its role in human physiology is unclear.Four major com- ponents of complement are designated by number, indi- cating chronological order of their discovery: C'1, C'2, C'3, and C'4 (11).A part of C'1 exists as a proenzyme which, under appropriate conditions, is converted to an active enzyme called C'1-esterase (12-18).Recent studies of Lepow and associates have shown that C'1 may be chromatographically resolved into three subcomponents, referred to as C'lq, C'lr, and C'ls (19).C'lq is an 11 S globulin, probably identical to the pro- tein that Mfiller-Eberhard and Kunkel called C'O (20) and Taranta, Weiss, and Franklin (21) described as participating in immune hemolysis.C'1r was not identi- fiable with previously described components of comple- ment and was proposed as a new component (19).C'1s was chromatographically and antigenically related to C'1-esterase (17-19, 22).Once activated, C'1-esterase can readily inactivate the fourth (C'4) and second (C'2) components of com- plement and hydrolyze several synthetic amino acid esters, most notably N-acetyl-L-tyrosine ethyl ester (12-15).Normal human serum contains an a2 globu- lin that inhibits these actions of C'1-esterase stoichio- metrically (15, 23-25).The action of this inhibitor was absent in hereditary angioneurotic edema (8).Procedure.Serum was separated from clotted blood by centrifugation at 0° C within an hour of venipuncture and stored at -600 C. Plasma was obtained by using disodium ethylenediamine tetraacetate (EDTA) as an anticoagulant to provide a final plasma concentration of 2204
DOI: 10.1172/jci105094
Authors: Virginia H. Donaldson, Fred S. Rosen
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W1991473086