SERPING1 Antibody, FITC conjugated

說明書

中文名稱：

SERPING1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA021086LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) SERPING1 Polyclonal antibody
Uniprot No.：

P05155
基因名：

SERPING1
別名：

C1 esterase inhibitor antibody; C1 Inh antibody; C1 inhibiting factor antibody; C1 inhibitor antibody; C1-inhibiting factor antibody; C1IN antibody; C1Inh antibody; C1NH antibody; complement component 1 inhibitor antibody; esterase inhibitor antibody; HAE1 antibody; HAE2 antibody; IC1_HUMAN antibody; Plasma protease C1 inhibitor antibody; Serine (or cysteine) proteinase inhibitor clade G member 1 antibody; serine/cysteine proteinase inhibitor clade G member 1 antibody; Serpin G1 antibody; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1 antibody; SERPING1 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Plasma protease C1 inhibitor protein (23-300AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
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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相關疾病：

Hereditary angioedema (HAE)
亞細胞定位：

Secreted.
蛋白家族：

Serpin family
數據庫鏈接：

HGNC: 1228

OMIM: 106100

KEGG: hsa:710

STRING: 9606.ENSP00000278407

UniGene: Hs.384598

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