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PMID: 5813121 Published · ppublish English Journal Article

Permeability-increasing activity in hereditary angioneurotic edema plasma. II. Mechanism of formation and partial characterization.

The Journal of clinical investigation ·Vol. 48 ·No. 4 ·1969-04-00 ·Pages 642-53

Donaldson VH, Ratnoff OD, Dias Da Silva W, Rosen FS

Abstract

Plasma from persons with hereditary angioneurotic edema readily developed the capacity to increase vascular permeability and to induce the isolated rat uterus to contract. Both activities resided in a small, heat-stable molecule that was apparently a polypeptide. Crude preparations of the polypeptide were inactivated during incubation with trypsin. They also failed to produce pain and erythema, but caused markedly increased vascular permeability in human skin. These characteristics differ from those of bradykinin, from which crude preparations of the polypeptide could also be distinguished by electrophoretic mobility and paper chromatographic behavior. Proof that the polypeptide is truly different from bradykinin must await its further purification. Histamine played no role in the activities observed. Although the enzymes functioning to release the permeability factor and kinin activities in hereditary angioneurotic edema plasma were not clearly defined, one or more plasma enzymes other than C'1 esterase presumably participated either in conjunction with C'1 esterase or in pari passu events to release the polypeptide mediating these activities.

MeSH Terms
Angioedema/blood,enzymology,genetics Animals Esterases/blood Female Guinea Pigs Humans Muscle Contraction Peptide Biosynthesis Peptide Hydrolases/blood Peptides/blood Permeability Skin/blood supply Uterus/physiology
Chemicals
Peptides Esterases Peptide Hydrolases
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Donaldson V H
Ratnoff O D
Dias Da Silva W
Rosen F S
References (40)
40 references, click to expand
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Article Info
Journal
The Journal of clinical investigation
Abbr.
J Clin Invest
ISSN
0021-9738
Published
1969-04-00
Pages
642-53
Language
English
Region
United States
NLM ID
7802877
PMCID
PMC322269
Subset
IM
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