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Human SERPING1 Protein expressed in Human - ABIN3119666
Zamolodchikov, Renné, Strickland: The Alzheimer's disease peptide β-amyloid promotes thrombin generation through activation of coagulation factor XII. in Journal of thrombosis and haemostasis : JTH 2016
C1INH prevented myocardial ischemia reperfusion injury in mice.
Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor.
Transgenic mice have been generated that express human C1 inhibitor mRNA and protein under the control of the human promoter and regulatory elements; C1nh production is demonstrated in mouse brain, spleen, liver, heart, kidney, and lung.
In vivo, C1 inhibitor (C1INH) reduces the number of viable bacteria in the blood and peritoneal fluid and accelerates killing of bacteria by blood neutrophils and peritoneal macrophages.
C1-inhibitor limits neointimal plaque formation and inflammation. This may involve blockade of complement activation, inhibition of leukocyte recruitment, and reduced triglyceride levels
In addition to the protective activities mediated via inhibition of the complement system, these studies indicate that C1INH also plays a direct role in suppression of leukocyte transmigration into reperfused tissue.
plasma-derived C1 esterase inhibitor concentrate has limited effect on house dust mite-induced allergic lung inflammation in mice
Data suggest that hereditary angioedema (HAE) may be caused by the deficiency of complement component 1 inhibitor protein (C1 (show PFDN4 Proteins)-inhibitor; SERPING1).
findings show that P. falciparum merozoites recruit C1-INH from human serum through an interaction with one of the merozoite surface proteins
PIC1 (show SUMO1 Proteins) inhibits the peroxidase activity of myeloperoxidase (show MPO Proteins) in cystic fibrosis (show S100A8 Proteins) sputum likely via an antioxidant mechanism.
like heparin, polyP is a naturally occurring cofactor for the C1s:C1-INH interaction and thus an important regulator of complement activation
Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease caused by mutations in the C1 inhibitor gene SERPING1. In the present study, the mutational spectrum of the SERPING1 gene in 19 patients of nine unrelated Swiss families were investigated.
Results identified a novel causative mutation in SERPING1, a deletion of two nucleotides on exon 3 in several affected members of two apparently unrelated families with a high frequency of hereditary angioedema.
Study presents the crystal structures of the serpin domain of C1-inhibitor in its active form by itself and in complex with dextran sulfate. The crystal structures and isothermic calorimetry studies show that dextran sulfate binds to multiple C1-inhibitor molecules with low affinity at C1-inhibitor's F1 helix and does not invoke an allosteric change.
Supraphysiological C1-INH concentrations have dose-dependent anticoagulant effects in human whole blood in vitro. At very high levels C1-INH also inhibits fibrinolysis. C1-INH abolished E.coli-induced coagulation measured by thromboelastometry.
This study represents the first Brazilian HAE cohort evaluated for SERPING1 gene mutations and it introduces the possibility to perform genetic analysis in case of need for differential diagnosis.
This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its protein inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. Deficiency of this protein is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform.
, c1 Inh
, plasma protease C1 inhibitor
, serine (or cysteine) peptidase inhibitor, clade G, member 1
, serpin peptidase inhibitor, clade G (C1 inhibitor), member 1, (angioedema, hereditary)
, serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
, plasma protease C1 inhibitor-like
, C1 Inh
, C1 esterase inhibitor
, C1-inhibiting factor
, complement component 1 inhibitor
, serine (or cysteine) proteinase inhibitor, clade G (C1 inhibitor), member 1
, serine (or cysteine) proteinase inhibitor, clade G, member 1
, serpin G1
, serine/cysteine proteinase inhibitor clade G member 1
, serine (or cysteine) proteinase inhibitor, clade G (C1 inhibitor), member 1, (angioedema, hereditary)
, serpin peptidase inhibitor, clade G, member 1
, complement component 1 inhibitor (angioedema, hereditary)
, factor XIIa inhibitor