Human Plasma protease C1 inhibitor(SERPING1) ELISA kit

Code CSB-EL021086HU
Size 96T,5×96T,10×96T
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Product Details

Target Name
serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
Alternative Names
C1 esterase inhibitor ELISA Kit; C1 Inh ELISA Kit; C1 inhibiting factor ELISA Kit; C1 inhibitor ELISA Kit; C1-inhibiting factor ELISA Kit; C1IN ELISA Kit; C1Inh ELISA Kit; C1NH ELISA Kit; complement component 1 inhibitor ELISA Kit; esterase inhibitor ELISA Kit; HAE1 ELISA Kit; HAE2 ELISA Kit; IC1_HUMAN ELISA Kit; Plasma protease C1 inhibitor ELISA Kit; Serine (or cysteine) proteinase inhibitor clade G member 1 ELISA Kit; serine/cysteine proteinase inhibitor clade G member 1 ELISA Kit; Serpin G1 ELISA Kit; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1 ELISA Kit; SERPING1 ELISA Kit
Abbreviation
SERPING1
Uniprot No.
Species
Homo sapiens (Human)
Sample Types
serum, plasma, tissue homogenates
Detection Range
18.75 ng/mL-1200 ng/mL
Sensitivity
4.7 ng/mL
Assay Time
1-5h
Sample Volume
50-100ul
Detection Wavelength
450 nm
Research Area
Blood Coagulation
Assay Principle
quantitative
Measurement
Competitive
Precision
Intra-assay Precision (Precision within an assay): CV%<8%      
Three samples of known concentration were tested twenty times on one plate to assess.  
Inter-assay Precision (Precision between assays): CV%<10%      
Three samples of known concentration were tested in twenty assays to assess.    
             
Linearity
To assess the linearity of the assay, samples were spiked with high concentrations of human SERPING1 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
  Sample Serum(n=4)  
1:200 Average % 99  
Range % 95-103  
1:400 Average % 94  
Range % 91-98  
1:800 Average % 95  
Range % 90-100  
1:1600 Average % 92  
Range % 85-96  
Recovery
The recovery of human SERPING1 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
Sample Type Average % Recovery Range  
Serum (n=5) 96 88-99  
EDTA plasma (n=4) 89 82-96  
             
             
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
ng/ml OD1 OD2 Average    
1200 0.090 0.091 0.091    
600 0.115 0.117 0.116    
300 0.166 0.175 0.171    
150 0.272 0.261 0.267    
75 0.466 0.455 0.461    
37.5 0.784 0.773 0.779    
18.75 1.020 1.110 1.065    
0 2.103 2.205 2.154    
Troubleshooting
and FAQs
Storage
Store at 2-8°C. Please refer to protocol.
Lead Time
3-5 working days after you place the order, and it takes another 3-5 days for delivery via DHL or FedEx
Description

This Human SERPING1 ELISA Kit was designed for the quantitative measurement of Human SERPING1 protein in serum, plasma, tissue homogenates. It is a Competitive ELISA kit, its detection range is 18.75 ng/mL-1200 ng/mL and the sensitivity is 4.7 ng/mL.

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Target Background

Function
(From Uniprot)
Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.
Gene References into Functions
  1. Study identified that peripheral mRNA expression levels of two complement genes (C5, SERPING1) made unique contributions to the variance in superior frontal cortical thickness. Vertex-wise maps of the association between gene expression levels and thickness across the cortex suggested that this relationship was especially strong with SERPING1 in the superior frontal region. PMID: 28758643
  2. Our analysis shows that SERPING1 mRNA is overexpressed in monocytes from HIV-1+ patients and the expression levels correlate positively with viral load and negatively with the CD4(+) T-cell count. PMID: 28889214
  3. A rare non-conservative missense mutation was newly identified in exon 9 of the PLG gene. PMID: 29548426
  4. plasma-derived C1 esterase inhibitor concentrate has limited effect on house dust mite-induced allergic lung inflammation in mice PMID: 29036225
  5. Data suggest that hereditary angioedema (HAE) may be caused by the deficiency of complement component 1 inhibitor protein (C1-inhibitor; SERPING1). PMID: 28595743
  6. findings show that P. falciparum merozoites recruit C1-INH from human serum through an interaction with one of the merozoite surface proteins PMID: 28484054
  7. PIC1 inhibits the peroxidase activity of myeloperoxidase in cystic fibrosis sputum likely via an antioxidant mechanism. PMID: 28135312
  8. like heparin, polyP is a naturally occurring cofactor for the C1s:C1-INH interaction and thus an important regulator of complement activation PMID: 27338096
  9. 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. PMID: 28194776
  10. 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. PMID: 27187751
  11. 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. PMID: 27818099
  12. 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. PMID: 27197075
  13. 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. PMID: 26812872
  14. Suggest that endogenous C1-inhibitor is likely involved in the regulation of complement activity in the myocardium following acute myocardial infarction. PMID: 26476955
  15. This family-based study provides the first evidence that multiple amino acid substitutions in SERPING1 could influence Hereditary angioedema due to C1-inhibitor deficiency phenotype PMID: 26895475
  16. the ratio of serum proteoglycan 4 to protease C1 inhibitor may be used for screening of early breast cancer. PMID: 26890881
  17. Enhancement of C1INH activity was not observed in the refractory septic shock patients, and the C1INH quantitative values were low. PMID: 26782794
  18. Novel mutations in the SERPING1 gene are linked to Hereditary Angioedema. PMID: 26535898
  19. Studied human C1INH and found at therapeutically relevant doses, it blocks malaria parasite invasion and cytoadhesion. PMID: 26347576
  20. SERPING1 mutations in Norwegian patients with Hereditary angioedema with C1 inhibitor deficiency, are reported. PMID: 26154504
  21. The levels of MASP-1 and MASP-1/C1-INH complexes are reduced in HAE patients compared with controls. Both MASP-1 and MASP-1/C1-INH complexes are related to the degree of complement C4 consumption PMID: 26371246
  22. SERPING1 is not a major genetic component of AMD or PCV in East Asians but is a genetic risk factor for AMD in Caucasians, providing evidence for an ethnic diversity in the genetic etiology of AMD. PMID: 25800435
  23. C1-inh polymers are present in the plasma of a subgroup of hereditary angioedema type I patients. PMID: 25369003
  24. Our case report demonstrates that children at a relatively early age can acquire the skills to practice intravenous administration of C1IHN concentrate independent of adult supervision. PMID: 25208595
  25. Suggest C1-inh polymers activate the FXII-dependent kallikrein-kinin system in hereditary angioedema. PMID: 25800206
  26. SERPING1 rs2511989 polymorphism may have a positive effect on the risk of age-related macular degeneration, especially among Caucasians. PMID: 25352749
  27. PBMCs extracted from controls, HAE-I and HAE-II patients presented identical methylation status of the SERPING1 promoter when analyzed by bisulphite sequencing PMID: 25053016
  28. A series of 22 different mutations was identified of C1NH gene in Italian patients with hereditary angioedema. PMID: 24456027
  29. study identified and characterized a new mutation in SERPING1 gene in a Spanish family with hereditary angioedema; the mutation (c.685 + 2 T > A) disrupts the donor splice site of intron 4 leading to the loss of exon 4 in mutant mRNA PMID: 24412907
  30. Dose escalation of nanofiltered C1 inhibitor (human) up to 2500 U was well tolerated and reduced attack frequency in the majority of hereditary angioedema patients. PMID: 24565773
  31. Relief of symptoms of Hereditary angioedema attacks is achieved faster with recombinant human C1INH compared with placebo as assessed by the treatment effect questionnaire and visual analog scale, with a positive safety profile. PMID: 24468257
  32. Women with recurrent angioedema unresponsive to antihistamines may have idiopathic angioedema or, more rarely, hereditary angioedema with F12 mutations. Both conditions may be provoked or aggravated by exogenous estrogens. PMID: 24262729
  33. SERPING1 does not play a significant role in the development of AU. PMID: 23966370
  34. This is the first report of a patient with hereditary angioedema due to a homozygous de novo null mutation of the C1NH gene. PMID: 23688413
  35. study established that the missense mutations of the C1INH gene are associated with a less severe form of hereditary angioedema PMID: 23265861
  36. Our study identified four novel mutations in the Slovenian HAE population, highlighting the heterogeneity of mutations in the SERPING1 gene causing C1 inhibitor deficiency and Hereditary angioedema . PMID: 23437219
  37. plays a role in activating coagulation during sepsis PMID: 23607270
  38. Ficolin-2, -3, and MAP-1 correlated negatively with the annual requirement of plasma derived C1-inhibitor concentrate. PMID: 23318225
  39. In the presence of heparin both C1-inh and AT are equally efficient inhibitors of the lectin pathway. PMID: 23399388
  40. Hereditary angioedema a rare inherited disease with C1 complement inhibitor protein expression. PMID: 22276768
  41. Identification of a novel and recurrent mutation in the SERPING1 gene in Sardinian patients with hereditary angioedema PMID: 23583915
  42. nonsense, frameshift, and mutations on Arg466 can cause lower level of C1 inhibitor antigen than missense and in-frame mutations; however, it does not affect severity of symptoms. PMID: 22994404
  43. identified a novel frame-shift mutation c.1391-1445del55 (p.v464fsx556) in exon 8 in a large Chinese family with hereditary angioedema type I PMID: 22882460
  44. The prevalence of the SERPING1missense mutation p.Arg444Cys was 39 out of 42 among Portuguese patients with hereditary angioedema. PMID: 23123409
  45. Letter: Report C1 inhibitor gene mutations in nine Japanese patients with hereditary angioedema. PMID: 22831796
  46. analysis of the 10-14 weeks' plasma samples showed the presence of a protein of mass 100.3 kDa that was elevated in the Down's syndrome group compared to the controls. This protein was identified as being plasma protease C1 inhibitor. PMID: 22456345
  47. [review] C1 INH is a multifaceted anti-inflammatory protein that exerts its effects through a variety of mechanisms that may or may not depend on protease inhibition via bonding or other non-covalent interactions with proteins, cell surfaces, or lipids. PMID: 21345278
  48. A deficiency of complement component 1 (C1) esterase inhibitor leads to overproduction of vasoactive kinins that cause angioedema. Review. PMID: 22456031
  49. Genetic variation in the promoter region of the SERPING1 gene may influence expression of complement component C1 inhibitor in age-related macular degeneration. PMID: 21852020
  50. The results of these studies provide new data about C1 inhibitor expression in hereditary angioedema patients and shed more light on the transcriptional regulation of the SERPING1 locus. PMID: 22001489

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Involvement in disease
Hereditary angioedema (HAE)
Subcellular Location
Secreted.
Protein Families
Serpin family
Database Links

HGNC: 1228

OMIM: 106100

KEGG: hsa:710

STRING: 9606.ENSP00000278407

UniGene: Hs.384598

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