HYPERACUTE rejection (HAR) of porcine organs is due to classical complement activation by naturally occurring human antibodies to the endothelium. Pigs that are transgenic for human regulators of complement (RCA) seem promising with respect to avoiding HAR, and their use in various studies has brought xenotransplantation closer to clinical reality. However, RCAs are usually expressed on the end...

The synthesis of the subunits of the C1 complex (C1q, C1s, C1r), and its regulator C1 inhibitor (C1-Inh) by human monocytes has been previously established. However, surface expression of these molecules by monocytes has not been shown. Using flow cytometry and antigen-capture enzyme-linked immunosorbent assay, we show here for the first time that, in addition to C1q, peripheral blood monocytes...

We present a new functional assay for the first complement component (C1) in plasma, based on its activation by inhibition of the C1-esterase inhibitor (C1-inh) when monospecific antiserum to C1-inh is added to the plasma. After maximal activation, we can determine the concentration of activated C1 by using an amidolytic rate assay with a chromogenic substrate. We have optimized the assay condi...

Cholesterol efflux capacity (CEC), the ability of high-density lipoprotein (HDL) cholesterol to accept from macrophages, has been linked cardiovascular events. Systemic lupus erythematosus (SLE) is characterized by consumption complement (C) proteins and associated with an increased risk disease. CEC reduced in SLE patients compared controls. In present work, our objective was analyze whether d...

Increased extracellular proteolysis because of unregulated activation of blood coagulation, complement, and fibrinolysis is observed in thrombosis, shock, and inflammation. In the present study, we have examined whether the plasma kallikrein-kinin system, the classical pathway of complement, and the fibrinolytic system could be inhibited by alpha 1-antitrypsin reactive site mutants. Wild-type a...

Patients with hereditary angioedema (HAE) present with recurrent, circumscribed, and self-limiting episodes of tissue or mucous membrane swelling caused by C1-inhibitor (CI-INH) deficiency. The estimated frequency of HAE is 1:50,000 persons. Distinguishing HAE from acquired angioedema (AAE) facilitates therapeutic interventions and family planning or testing. Patients with HAE benefit from trea...

We report on an angioedema patient with a genetic defect in complement 1 inhibitor, manifesting migraine-like episodes of headache, effective prophylaxis with Danazol, and triptan for a treatment of acute clinical episode. The patient was 44-yr-old Korean man with abdominal pain and headache, who was brought into the Emergency Department of Seoul National University Hospital, Seoul. He suffered...

Th e complement system is a crucial part of the body’s defense mechanism against bacterial infection and is vital for protecting individuals against any disturbance of homeostasis associated with the appearance of foreign antigens [1]. Activation of the complement system can be initiated through the classic pathway antigen-antibody complexes or through the alternative and lectin pathways by non...