Reactivity | HuSpecies Glossary |
Applications | Bioactivity |
Format | Carrier-Free |
Details of Functionality | Measured by its binding ability in a functional ELISA. When Recombinant Human Complement Factor H-related 1/CFHR1 is
immobilized at 1 μg/mL
(100 μL/well), the concentration of Biotinylated Recombinant Mouse Complement Component C3d
that produces 50% of the optimal binding response is 40-240 ng/mL. |
Source | Mouse myeloma cell line, NS0-derived human Complement Factor H-related 1/CFHR1 protein Glu19-Ala328, with a C-terminal 10-His tag |
Accession # | |
N-terminal Sequence | Glu19 |
Protein/Peptide Type | Recombinant Proteins |
Purity | >95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining. |
Endotoxin Note | <0.10 EU per 1 μg of the protein by the LAL method. |
Dilutions |
|
Theoretical MW | 37 kDa. Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors. |
SDS-PAGE | 39-48 kDa, reducing conditions |
Storage | Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
|
Buffer | Lyophilized from a 0.2 μm filtered solution in Tris and NaCl with Trehalose. |
Purity | >95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining. |
Reconstitution Instructions | Reconstitute at 500 μg/mL in water. |
Complement factor H-related protein 1 (CFHR1) is a ~40 kDa secreted, primarily homodimeric member of the factor H family of glycoproteins (1). CFHR1 is produced by hepatocytes and circulates as two differentially glycosylated isoforms. The human complement factor H protein family consists of the complement and immune regulators factor H, the factor H-like protein 1 (FHL-1) and five factor H-related proteins (FHR-1 to -5) (2). The genes of this family are located on human chromosome 1q32, which is known as the regulator of complement activation (RCA) gene clusters (3). CFHRs are exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. CFHR1 contains 5 SCRs. Although they are considered group 1 CFHRs based on conserved N-termini (2), compared to CFHR1, human CFHR2 and CFHR5 show 67.5% and 34.5% aa identity, respectively. All CFHRs, including CHFR1, are capable of binding complement factor C3b, discriminate between self and non-self cell surfaces, and have been proposed to deregulate complement activation by inhibiting interaction of CFH with C3b (2). CFHR1 inhibits complement C5 convertase activity (4). A common CFHR1/CFHR3 genetic deletion has been implicated as being protective against age-related macular degeneration (5) and nephropathy (6). The same deletion is associated with development of factor H auto-antibodies and susceptibility to atypical hemolytic uremic syndrome due to the deletion of CFHR1 (7-8). Additionally, CFHR1 is recruited to the surface of pathogens which is suggested to result in evasion of host complement attack (9).
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