SRP6317
C1 Esterase inhibitor from human plasma
≥95% (SDS-PAGE)
Synonim(y):
C1-inhibiting factor, Complement C1 esterase inhibitor, Esterase inhibitor C-1, Plasma protease C1 inhibitor, Serpin G1
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About This Item
Polecane produkty
pochodzenie biologiczne
human
Próba
≥95% (SDS-PAGE)
Postać
frozen liquid
masa cząsteczkowa
100 kDa
opakowanie
pkg of 1 mg
numer dostępu UniProt
Warunki transportu
dry ice
temp. przechowywania
−70°C
informacje o genach
human ... Serpin G1(710)
Opis ogólny
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.
Działania biochem./fizjol.
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.
Postać fizyczna
Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl.
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Kod klasy składowania
11 - Combustible Solids
Klasa zagrożenia wodnego (WGK)
WGK 3
Temperatura zapłonu (°F)
Not applicable
Temperatura zapłonu (°C)
Not applicable
Certyfikaty analizy (CoA)
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Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.
In vivo biosynthesis of endogenous and of human C1 inhibitor in transgenic mice: tissue distribution and colocalization of their expression.
Journal of Immunology, 169(10), 5948-5954 (2002)
Genome biology, 7(5), 216-216 (2006-06-02)
Serpins are a broadly distributed family of protease inhibitors that use a conformational change to inhibit target enzymes. They are central in controlling many important proteolytic cascades, including the mammalian coagulation pathways. Serpins are conformationally labile and many of the
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