ELISA/assay

Human Complement Factor I ELISA Kit

MBS721578

48-Strip-Wells-(Comp

470 USD

ELISA Kit

Human Complement Factor I ELISA Kit

Complement Factor I

complement factor I preproprotein; Complement factor I; complement factor I; C3b-inactivator; C3B/C4B inactivator; complement component I; light chain of factor I; Konglutinogen-activating factor; complement factor I heavy chain; complement control protein 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

This assay has high sensitivity and excellent specificity for detection of CFI. No significant cross-reactivity or interference between CFI and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between CFI and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Assay Type: Competitive or Sandwich. Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Sensitivity: 0.1 ng/mL.

Intended Uses: This CFI ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human CFI. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

Immunology

Principle of the assay: CFI ELISA kit applies the quantitative sandwich enzyme immunoassay technique. The microtiter plate has been pre-coated with a monoclonal antibody specific for CFI. Standards or samples are then added to the microtiter plate wells and CFI if present, will bind to the antibody pre-coated wells. In order to quantitatively determine the amount of CFI present in the sample, a standardized preparation of horseradish peroxidase (HRP)-conjugated polyclonal antibody, specific for CFI are added to each well to "sandwich" the CFI immobilized on the plate. The microtiter plate undergoes incubation, and then the wells are thoroughly washed to remove all unbound components. Next, substrate solutions are added to each well. The enzyme (HRP) and substrate are allowed to react over a short incubation period. Only those wells that contain CFI and enzyme-conjugated antibody will exhibit a change in color. The enzyme-substrate reaction is terminated by addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The CFI concentration in each sample is interpolated from this standard curve.

119392081

NP_000195.2

NM_000204.3

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: Secreted, signal peptide; Secreted; EC 3.4.21.45; Protease. Chromosomal Location of Human Ortholog: 4q25. Cellular Component: extracellular space; membrane; extracellular region; nucleus. Molecular Function: metal ion binding; serine-type endopeptidase activity; 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

48-Strip-Wells-(Competitive)

470 USD

48-Strip-Wells-(Sandwich)

470

96-Strip-Wells-(Competitive)

675

96-Strip-Wells-(Sandwich)

675