Product Type
Antibody
Description
The monoclonal antibody MBI-1 recognizes complement factor I (CFI). CFI is a protein (serine protease) of the complement system, also known as C3b/C4f inactivator. It is a protein encoded by the CFI gene and is located on chromosome 4. It regulates the activation of complement by lysing cell binding or liquid phase. C3b and C4b. CFI is mainly synthesized in the liver and is initially secreted as a single 88 kDa gene product. Then, the precursor protein is cleaved by furin to produce a mature CFI protein, which is the two residues of the heavy chain (residues 19-335, 51 kDa) and the light chain (residues 340-583, 37 kDa). Sulfur-linked dimer. Only mature proteins are active. Genetic polymorphisms in CFI have been observed (variants R201S, R406H, R502L). CFI deficiency can lead to uncontrolled activation of the complement alternative pathway, resulting in a decrease in the level of complement component 3 (C3) in plasma, and is associated with repeated bacterial infections in children; recently, mutations in the CFI gene have been shown to be associated with the development of hemolytic uremic syndrome Hemolytic uremic syndrome is also a kidney disease caused by uncontrolled complement activation. Monoclonal antibodies can be used to detect CFI levels in serum and plasma.
Disease
Infectious diseases, Nephrology
Size
100 µg
Immunogen
Complement factor I
Species
Human
Clonality
Monoclonal
Clone Number
MBI-1
Buffer
PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.
Application
Immuno assays, Western blot
Application Notes
For Western blots, the diluent to be used depends on the detection system used. It is recommended that users test reagents and determine their own optimal dilution. The typical initial working dilution is 1:50.
Storage Instructions
Product should be stored at 4°C. Under recommended storage conditions, product is stable for at least one year.
Alternative Names
CFI; AHUS3; ARMD13; C3BINA; C3b-INA; FI; IF; KAF; complement factor I
