ELISA/assay

Human CFI (Complement Factor I) ELISA Kit

MBS2504645

24-Strip-Wells

215 USD

ELISA Kit

Human CFI (Complement Factor I) ELISA Kit

[CFI]

[complement factor I preproprotein; Complement factor I; complement factor I; C3B/C4B inactivator; C3b-inactivator; Konglutinogen-activating factor; complement component I; complement control protein factor I; complement factor I heavy chain; light chain of factor I; complement factor I; C3B/C4B inactivatorCleaved into the following 2 chains:Complement factor I heavy chain; Complement factor I light chain]

[CFI]

[CFI; CFI; FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA; IF]

CFAI_HUMAN

Human

583

This kit recognizes natural and recombinantHumanCFI. No significant cross-reactivity or interference between HumanCFI and analogues was observed.

Store at 4 degree C.

Typical Testing Data/Standard Curve (for reference only)

Samples: Serum, Plasma, Biological Fluids. Assay Type: Sandwich. Detection Range: 2.344-150ng/mL. Sensitivity: Min: 1.406ng/mL; Max: 150ng/mL

Intended Uses: This ELISA kit applies to the invitro quantitative determination of HumanCFIconcentrations in serum, plasma and other biological fluids. Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to CFI. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for CFIand Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain CFI, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of CFI.You can calculate the concentration of CFIin the samples by comparing the OD of the samples to the standard curve.

119392081

NP_000195.2

NM_000204.3

P05156

65,750 Da

Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Immune System Pathway (106386); Innate Immune System Pathway (106387); Regulation Of Complement Cascade Pathway (576254); Staphylococcus Aureus Infection Pathway (172846); Staphylococcus Aureus Infection Pathway (171867)

This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene. [provided by RefSeq, Jul 2008]

CFI: Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3). An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency). CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections. Belongs to the peptidase S1 family. Protein type: EC 3.4.21.45; Secreted, signal peptide; Protease; Secreted. Chromosomal Location of Human Ortholog: 4q25. Cellular Component: extracellular space; membrane; extracellular region; nucleus. Molecular Function: serine-type endopeptidase activity; metal ion binding; scavenger receptor activity. Biological Process: receptor-mediated endocytosis; regulation of complement activation; innate immune response; proteolysis; complement activation, classical pathway. Disease: Macular Degeneration, Age-related, 13; Complement Factor I Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 3

24-Strip-Wells

215 USD

48-Strip-Wells

410

96-Strip-Wells

490

5x96-Strip-Wells

2040

10x96-Strip-Wells

3590