C1-INH Antibody (Polyclonal)

A goat antiserum raised against human C1 Inhibitor protein.

> 40 mg/mL

Goat

Short term (30 days) 4˚C. Long term at or below –20˚C.

C1-inhibitor is a multi-specific protease inhibitor present in human plasma and serum. Normal human serum shows a concentration of approximately 200 µg/mL. C1-inhibitor is a glycoprotein with an approximate molecular weight of 110 kD. This protein contains many glycosylation sites, so the molecular weight varies depending on the quantity of sites that are glycosylated. Among the proteins regulated by C1-inhibitor are C1s and C1r of the complement complex. Regulation and inactivation of C1s and C1r effectively limits the consumption of complement components C2 and C4. Genetic deficiencies of C1-inhibitor are of two types: functional deficiencies (protein is present but not functional) and antigen deficiencies (protein is not present, or more commonly, present at a low level). Either of these may lead to life threatening hereditary angioedema (HAE).