Cleaved-CFB (K260) Antibody

說明書

中文名稱：

Cleaved-CFB (K260)兔多克隆抗體
貨號：

CSB-PA040288
規格：

￥1090
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P00751
基因名：

CFB
別名：

AHUS4 antibody; B factor properdin antibody; BF antibody; BFD antibody; C3 proaccelerator antibody; C3 proactivator antibody; C3/C5 convertase antibody; CFAB antibody; CFAB_HUMAN antibody; CFB antibody; Complement Factor B antibody; Complement factor B Bb fragment antibody; FB antibody; FBI12 antibody; GBG antibody; Glycine rich beta glycoprotein antibody; Glycine-rich beta glycoprotein antibody; H2 Bf antibody; H2BF antibody; PBF2 antibody; Properdin factor B antibody
宿主：

Rabbit
反應種屬：

Human,Mouse
免疫原：

Synthesized peptide derived from the Internal region of Human Factor B Bb.
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度：

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

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
產品提供形式：

Liquid
應用范圍：

WB, ELISA

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
ELISA	1:20000

Protocols：

Western Blotting (WB) Protocol
ELISA Protocol
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：

Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B-lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes.
基因功能參考文獻：
1. Study demonstrated that a novel complotype composed of CFB (rs4151667) in combination with CFB (rs641153) and CFH(rs800292) is strongly associated with complement activation and age-related macular degeneration status. PMID: 27241480
2. target sequencing of age-related macular degeneration (AMD) susceptibility genes identified enrichment of low-frequency coding variants in CETP, C2 and CFB are associated with AMD susceptibility in the Japanese population PMID: 28173125
3. Heterozygous variants in the CFB gene can be pathogenic and associated with immune-complex diffuse membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome PMID: 28210841
4. The complement activation factors Bb, C3a, C5a, and MAC were increased significantly in early-onset severe pre-eclampsia (EOSPE) (all P<.01) and late-onset severe pre-eclampsia (LOSPE). (P value: .027, <.001, .001, and <.001, respectively) compared with E/L-control. C1q and C4d were increased significantly in LOSPE (P value: .003 and .014, respectively) compared with L-control. PMID: 27461873
5. These results suggest (i) apparent allelic heterogeneity in CFB and genetic heterogeneity in SLC44A4 across different ethnic groups; (ii) shared ulcerative colitis genetic etiological factors among Asians PMID: 27759029
6. Neutralization of the complement factor C3-dependent antichlamydial activity was dependent on the proteolytic activity of Chlamydia trachomatis CPAF and correlated with the CPAF-mediated degradation of complement factor C3 and factor B. PMID: 27436813
7. Our results revealed a significant association of CFB with non-infectious uveitis, particularly predisposed to VKH disease. Genetic differences for uveitis could be gender-specific. PMID: 26671509
8. There is a link between phenotype BF SS07 and allotype BF*S07 with aCl-IgM in systemic lupus erythematosus patients; BF*F allotype could be considered a marker of protection against the development of antiphospholipid antibodies in these patients. PMID: 26537423
9. The Relationship of Longitudinal Levels of Complement Bb During Pregnancy with Preeclampsia PMID: 26510395
10. P serum level expression could be a reliable clinical biomarker to identify patients with underlying surface alternative pathway C5 convertase dysregulation. PMID: 26660535
11. CFB is downregulated in non-small cell lung cancer patients compared to those with benign lung disease or no lung disease. PMID: 26908325
12. These results suggest that a maternal immune response through complement fB might play a role in the development of preeclampsia, particularly in African-American patients. PMID: 25604034
13. mutation results in atypical hemolytic uremic syndrome PMID: 24906628
14. individuals with the chronic hepatitis B (CHB) risk genotype CC of rs12614 had significantly lower CFB concentrations than those carrying one or two rs12614 T alleles (CT or TT carriers) both in normal populations and CHB patients PMID: 25802187
15. Complement factor B is a novel biomarker candidate for pancreatic ductal adenocarcinoma. PMID: 25057901
16. complement factor B has an important role in the etiology of familial C3 glomerulonephritis PMID: 25758434
17. A mutation in complement factor B was associated with a case of C3 glomerulonephritis. PMID: 25532781
18. The C2 and CFB gene variants were shown to be associated with polypoidal CNV. Typical PCV was not associated with variants in these genes. PMID: 24965207
19. Complement factor B is potently upregulated locally in inflammatory bowel disease in addition to having a possible central role in systemic complement activation. PMID: 24739633
20. studied the functional consequences of 10 FB genetic changes recently identified from different atypical hemolytic uremic syndrome cohorts PMID: 24652797
21. The rs547154, rs641153, and rs12614 SNPs were not associated with age-related macular degeneration development in Greek patients. PMID: 24519512
22. ARMS2 and C3 are major contributors to advanced age-related macular degeneration in Mexican patients, while the contributions of CFH, C2, and CFB are minor to those of other populations. PMID: 24453474
23. The CFB (R32Q) polymorphism was associated with age-related macular degeneration characterized by small drusen only, and appeared to be protective of large drusen. PMID: 23373431
24. we have assessed the relationship between GA and previously identified AMD-associated variants of genes (CFH, CFB, C3, FHR1, FRH3, and ARMS2/HTRA). PMID: 24557084
25. Describe electroluminescent platform for profiling complement factor B in complement cascade activation. PMID: 24287422
26. CFH-rs800292 and CFB-rs1048709 are associated with the presence of diabetic retinopathy, which strengthens the concept that complement system plays an important role in the pathogenesis of DR PMID: 23864767
27. Eculizumab is effective therapy for atypical hemolytic uremic syndrome associated with factor B mutations. PMID: 23624872
28. Gain of function mutation in factor B is associated with recurrence in adult renal transplant recipients with atypical hemolytic and uremic syndrome. PMID: 23356914
29. Gene variants in CFH and C2/CFB contribute to age related macular degeneration in the Chinese population. PMID: 23233260
30. CFH, ARMS2, and CFB AMD-risk alleles are consistently associated with the disease, even in ethnic groups with a complex admixture of ancestral populations such as Mexican mestizos. PMID: 23112567
31. Increased activation of the alternative complement pathway in vitreous was controlled by disease stage and genetic variation in the complement pathway, supporting a role for complement activation in macular degeneration disease pathogenesis. PMID: 22930722
32. This meta-analysis provides evidence for an association between C2/CFB polymorphisms and age-related macular degeneration. PMID: 22869612
33. Our results revealed an association between Anterior uveitis and Complement Factor B-rs1048709. PMID: 22714898
34. significantly increased levels in patients with antiphospholipid antibodies or primary antiphospholipid syndrome PMID: 22234447
35. Women who were obese with levels of Bb or C3a in the top quartile were 10.0 (95% confidence interval, 3.3-30) and 8.8 (95% confidence interval, 3-24) times, respectively, more likely to develop preeclampsia compared with the referent group. PMID: 22542119
36. In conclusion, the genetic effect of C2, CFB and C3 polymorphisms, which are known to be important for AMD in Caucasian, were not significant in the Korean population. PMID: 22273503
37. C2/CFB variants play a protective role in the risk of developing neovascular AMD and PCV in the Japanese. PMID: 22232432
38. This study did not detect an association between individual age-related macular degeneration risk genotypes and the putatively protective macular pigments, or serum concentrations of its constituent carotenoids PMID: 21816153
39. Access to the complement factor B scissile bond is facilitated by association of factor B with C3b protein. PMID: 21862585
40. the concept of a functional complotype (combination of C3(R102G), factor B (fB(R32Q), and factor H (fH(V62I) polymorphisms) in defining complement activity in an individual, influencing susceptibility to alternative pathway-driven disease. PMID: 21555552
41. Complement factor B polymorphism 32W protects against age-related macular degeneration. PMID: 21541267
42. the association with the known genetic susceptibility loci CFH, HTRA1, and AMRS2 were confirmed, and a risk-conferring polymorphism in one new locus, LRP5, was identified. PMID: 21282580
43. These studies show that the acquisition of fH to the S. aureus surface inhibits complement-mediated opsonization via disruption of the alternative pathway convertase. PMID: 21163532
44. CFB 32W (rs12614; T) protects against age-related macular degeneration compared to the common CFB 32R. The protective effect is less strong than CFB 32Q. Knowledge of both rs641153 and rs12614 is required to predict the amino acid at residue 32. PMID: 21541267
45. Studies indicate that mutations or polymorphisms in complement genes C3 and factor B are genetic risk factors contributing hemolytic uremic syndrome. PMID: 20837143
46. crystal structure of C3bB at 4 A and complex with factor D at 3.5 A; data show how factor B binding to C3b forms open "activation" state of C3bB; Factor D binds open conformation of factor B through a site distant from the catalytic center PMID: 21205667
47. Reduced expression of alpha-2 macroglobulin and complement factor B was detected in sera of patients with nasopharyngeal carcinoma. PMID: 20575108
48. This study showed that CFH was more likely to be age-related macular(AMD) susceptibility gene, and none of the other C2, CFB, and C3 genes were associated with AMD in a white population. PMID: 20523265
49. The polypoidal choroidal vasculopathy (PCV) phenotype in Caucasian patients is associated with the major alleles/genotypes in the age-related macular degeneration (AMD)-associated loci, suggesting that PCV and AMD are genetically similar. PMID: 20378180
50. Case Report: 8-year-old girl diagnosed with atypical hemolytic uremic syndrome (aHUS) with a complement factor B (CFB) gene mutation. PMID: 20108004
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相關疾病：

Hemolytic uremic syndrome atypical 4 (AHUS4); Complement factor B deficiency (CFBD)
亞細胞定位：

Secreted.
蛋白家族：

Peptidase S1 family
數據庫鏈接：

HGNC: 1037

OMIM: 138470

KEGG: hsa:629

STRING: 9606.ENSP00000416561

UniGene: Hs.69771

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