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Safety Information

SRP6317

Sigma-Aldrich

C1 Esterase inhibitor from human plasma

≥95% (SDS-PAGE)

Synonym(s):

C1-inhibiting factor, Complement C1 esterase inhibitor, Esterase inhibitor C-1, Plasma protease C1 inhibitor, Serpin G1

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About This Item

UNSPSC Code:
12352204
NACRES:
NA.32

biological source

human

Assay

≥95% (SDS-PAGE)

form

frozen liquid

mol wt

100 kDa

packaging

pkg of 1 mg

UniProt accession no.

P05155

shipped in

dry ice

storage temp.

−70°C

Gene Information

human ... Serpin G1(710)

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.

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.

Physical form

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

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Listings

Regulatory Listings are mainly provided for chemical products. Only limited information can be provided here for non-chemical products. No entry means none of the components are listed. It is the user’s obligation to ensure the safe and legal use of the product.

JAN Code

SRP6317-1MG:

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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In vivo biosynthesis of endogenous and of human C1 inhibitor in transgenic mice: tissue distribution and colocalization of their expression.
Vinci G
Journal of Immunology, 169(10), 5948-5954 (2002)
Ruby H P Law et al.
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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