Human factor B,BF ELISA Kit

Code CSB-E09447h
Size 96T,5×96T,10×96T
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Product Details

Target Name
complement factor B
Alternative Names
AHUS4 ELISA Kit; B factor properdin ELISA Kit; BF ELISA Kit; BFD ELISA Kit; C3 proaccelerator ELISA Kit; C3 proactivator ELISA Kit; C3/C5 convertase ELISA Kit; CFAB ELISA Kit; CFAB_HUMAN ELISA Kit; CFB ELISA Kit; Complement Factor B ELISA Kit; Complement factor B Bb fragment ELISA Kit; FB ELISA Kit; FBI12 ELISA Kit; GBG ELISA Kit; Glycine rich beta glycoprotein ELISA Kit; Glycine-rich beta glycoprotein ELISA Kit; H2 Bf ELISA Kit; H2BF ELISA Kit; PBF2 ELISA Kit; Properdin factor B ELISA Kit
Abbreviation
CFB
Uniprot No.
Species
Homo sapiens (Human)
Sample Types
serum, plasma
Detection Range
1.56 ng/mL-100 ng/mL
Sensitivity
0.39 ng/mL
Assay Time
1-5h
Sample Volume
50-100ul
Detection Wavelength
450 nm
Research Area
Immunology
Assay Principle
quantitative
Measurement
Sandwich
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 BF in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
SampleSerum(n=4)
1:1000Average %97
Range %89-105
1:2000Average %89
Range %85-98
1:4000Average %93
Range %90-97
1:8000Average %96
Range %89-101
Recovery
The recovery of human BF 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 TypeAverage % RecoveryRange
Serum (n=5) 9688-101
EDTA plasma (n=4)9791-106
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
ng/mlOD1OD2AverageCorrected
1002.254 2.304 2.279 2.149
501.816 1.853 1.835 1.705
251.507 1.439 1.473 1.343
12.51.042 1.072 1.057 0.927
6.250.813 0.802 0.808 0.678
3.120.492 0.485 0.489 0.359
1.560.302 0.300 0.301 0.171
00.132 0.128 0.130
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 CFB ELISA Kit was designed for the quantitative measurement of Human CFB protein in serum, plasma. It is a Sandwich ELISA kit, its detection range is 1.56 ng/mL-100 ng/mL and the sensitivity is 0.39 ng/mL.

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Target Background

Function
(From Uniprot)
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.
Gene References into Functions
  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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Involvement in disease
Hemolytic uremic syndrome atypical 4 (AHUS4); Complement factor B deficiency (CFBD)
Subcellular Location
Secreted.
Protein Families
Peptidase S1 family
Database Links

HGNC: 1037

OMIM: 138470

KEGG: hsa:629

STRING: 9606.ENSP00000416561

UniGene: Hs.69771

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