An autoantibody to C1-inhibitor recognizes the reactive center of the inhibitor.
An autoantibody to C1-inhibitor produced a clinical disorder resembling that of patients with hereditary angioneurotic edema. The antibody could not interact with C1-inhibitor after exposure to synthetic peptides representing the primary structure of the reactive center region of the protein. Therefore the antibody recognized this domain of the inhibitor, and it probably impaired the function of C1-inhibitor by altering its conformational properties.
Donaldson
V H
VH
Children's Hospital Research Foundation, Cincinnati, OH 45229-3039, USA.
Wagner
C J
CJ
Davis
A E
AE
D000595:Amino Acid Sequence; D000799:Angioedema / Q000235:genetics / Q000276:immunology; D001323:Autoantibodies; D001665:Binding Sites; D003174:Complement C1 Inactivator Proteins / Q000737:chemistry* / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G; D008969:Molecular Sequence Data; D010446:Peptide Fragments / Q000737:chemistry / Q000276:immunology; D010455:Peptides / Q000138:chemical synthesis / Q000276:immunology
D001323:Autoantibodies; D003174:Complement C1 Inactivator Proteins; D007074:Immunoglobulin G; D010446:Peptide Fragments; D010455:Peptides