C1 ESTERASE INHIBITORHUMAN PLASMA

Code: SRP6317-1MG D2-231

Biochem/physiol Actions

C1 esterase inhibitor functions as a serine proteinase inhibitor. The concentration of C1 esterase inhibitor protein is reduced to 10-30% of normal in...


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€521.30 1MG
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Biochem/physiol Actions

C1 esterase inhibitor functions as a serine proteinase inhibitor. The concentration of C1 esterase inhibitor protein is reduced to 10-30% of normal in patients with angioedema secondary to C1 esterase inhibitor deficiency (85% of patients with Hereditary Angioedema (HAE)); in 15% of patients with HAE, the concentrations of the inhibitor protein is normal but function is markedly reduced. C1 esterase inhibitor deficiency is a rare condition resulting in facial swelling and abdominal cramping. Usually the condition is hereditary, though it may also occur when the protein is non-functional. C1 esterase inhibitor deficiencies also disturb the fibrinolytic system, the intrinsic coagulation pathway and the complement pathway.

General description

C1 esterase inhibitor is a single chain glycoprotein which inhibits C1, C1r, C1s, plasma kallikrein, factors XIa, XIIa and plasmin of the blood clotting system. It is present in the plasma at 16-33 mg/100mL. It is part of the serpin family.

Physical form

Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.

assay≥95% (SDS-PAGE)
biological sourcehuman
formfrozen liquid
Gene Informationhuman ... Serpin G1(710)
mol wt100 kDa
packagingpkg of 1 mg
shipped indry ice
storage temp.−70°C
UniProt accession no.P05155
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