Product Type
Antibody
Description
The monoclonal antibody JHD-7.10.1 reacts with human complement factor H related proteins 1 and 2 (CFHR1/2). In humans, the complement factor H protein family consists of complement factor H, factor H-like protein 1, and five factor H-related proteins (called 1 to 5). All members of this group are mainly expressed in the liver and then secreted into the plasma. The secreted form of these proteins consists only of repetitive protein domains called short consensus repeats (SCR). The CFHR1 protein is composed of 5 SCR domains and circulates in plasma in two differentially glycosylated subtypes (37 kDa and 43 kDa). CFHR-2 consists of four SCRs, which are present in human plasma in non-glycosylated (24 kDa) and 29 kDa glycosylated forms. So far, little is known about the biological functions of CFHR-1 and CFHR-2 proteins, but recent studies have shown that CFHR1 is a complement regulator that can block the activity of C5 convertase and the assembly of terminal components and membrane insertion . In addition, genetic variations in the CFHR1 gene are associated with several diseases, such as atypical hemolytic uremic syndrome and age-related macular degeneration. Due to the high degree of amino acid sequence homology, monoclonal antibodies can cross-react with CFHR1 and 2. This antibody is suitable for immunoassay and Western blot applications.
Disease
Autoimmunity, Nephrology
Size
100 µg
Immunogen
Synthetic peptide representing the last 16 C-terminal (NKTVAVRTLDPERLGR) residues of C3c alpha domain 1 coupled onto PPD (purified protein derivate, Statens Serum Institut)
Species
Human
Clonality
Monoclonal
Clone Number
JHD-7.10.1
Buffer
PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.
Application
Immuno assays, Western blot
Application Notes
For immunoassays and western blotting, 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
Complement factor H related protein; CFHR
