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Merck
모든 사진(1)

주요 문서

SRP6317

Sigma-Aldrich

C1 Esterase inhibitor from human plasma

≥95% (SDS-PAGE)

동의어(들):

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 코드:
12352204
NACRES:
NA.32

생물학적 소스

human

분석

≥95% (SDS-PAGE)

형태

frozen liquid

분자량

100 kDa

포장

pkg of 1 mg

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−70°C

유전자 정보

human ... Serpin G1(710)

일반 설명

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.

생화학적/생리학적 작용

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.

물리적 형태

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


시험 성적서(COA)

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문서 라이브러리 방문

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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