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  4. Recombinant Human Plasma protease C1 inhibitor (SERPING1)

Recombinant Human Plasma protease C1 inhibitor (SERPING1)

  • 货号：
    CSB-YP021086HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP021086HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP021086HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 货号：
    CSB-BP021086HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP021086HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 别名：
    C1 esterase inhibitor; C1 Inh; C1 inhibiting factor; C1 inhibitor; C1-inhibiting factor; C1IN; C1Inh; C1NH; complement component 1 inhibitor; esterase inhibitor; HAE1; HAE2; IC1_HUMAN; Plasma protease C1 inhibitor; Serine (or cysteine) proteinase inhibitor clade G member 1; serine/cysteine proteinase inhibitor clade G member 1; Serpin G1; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1; SERPING1
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Mature Protein
  • 表达区域：
    23-500
  • 氨基酸序列
    NPNATSSS SQDPESLQDR GEGKVATTVI SKMLFVEPIL EVSSLPTTNS TTNSATKITA NTTDEPTTQP TTEPTTQPTI QPTQPTTQLP TDSPTQPTTG SFCPGPVTLC SDLESHSTEA VLGDALVDFS LKLYHAFSAM KKVETNMAFS PFSIASLLTQ VLLGAGENTK TNLESILSYP KDFTCVHQAL KGFTTKGVTS VSQIFHSPDL AIRDTFVNAS RTLYSSSPRV LSNNSDANLE LINTWVAKNT NNKISRLLDS LPSDTRLVLL NAIYLSAKWK TTFDPKKTRM EPFHFKNSVI KVPMMNSKKY PVAHFIDQTL KAKVGQLQLS HNLSLVILVP QNLKHRLEDM EQALSPSVFK AIMEKLEMSK FQPTLLTLPR IKVTTSQDML SIMEKLEFFD FSYDLNLCGL TEDPDLQVSA MQHQTVLELT ETGVEAAAAS AISVARTLLV FEVQQPFLFV LWDQQHKFPV FMGRVYDPRA
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Hereditary angioedema (HAE)
  • 亚细胞定位：
    Secreted.
  • 蛋白家族：
    Serpin family
  • 数据库链接：

    HGNC: 1228

    OMIM: 106100

    KEGG: hsa:710

    STRING: 9606.ENSP00000278407

    UniGene: Hs.384598