Recombinant Human CFHR1 Protein (C-6His)

Name: Recombinant Human CFHR1 Protein (C-6His)
Brand: Beta LifeScience
SKU: BL-0556NP
Availability: InStock

BL-0556NP: Greater than 95% as determined by reducing SDS-PAGE. (QC verified)

Collections: Other recombinant proteins, Recombinant proteins

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Product Overview

Description	Recombinant Human Complement Factor H-Related 1 is produced by our Mammalian expression system and the target gene encoding Glu19-Arg330 is expressed with a 6His tag at the C-terminus.
Accession	Q03591
Synonym	Complement Factor H-Related Protein 1; FHR-1; H Factor-Like Protein 1; H-Factor-Like 1; H36; CFHR1; CFHL; CFHL1; CFHL1P; CFHR1P; FHR1; HFL1; HFL2
Gene Background	Complement Factor H-Related 1 (CFHR1) is a 43 kDa secreted member of the factor H family of glycoproteins. 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 (CFHR-1 to -5). Members of the H-related protein family are exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. FHR1 is produced by hepatocytes and circulates as two differentially glycosylated isoforms (37 kDa and 43 kDa). Mature human FHR1 is 312 amino acids in length. It contains five, approximately 60 aa SCRs that basically constitute the entire molecule. FHR1 may play a role in complement regulation, lipid metabolism and lipoprotein complexes that bind PMNs to LPS.
Molecular Mass	36.78 KDa
Apmol Mass	39-43 KDa, reducing conditions
Formulation	Lyophilized from a 0.2 μm filtered solution of 20mM PB, 150mM NaCl, 2mM EDTA, pH 7.4.
Endotoxin	Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test.
Purity	Greater than 95% as determined by reducing SDS-PAGE. (QC verified)
Biological Activity	Not tested
Reconstitution	Always centrifuge tubes before opening. Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
Storage	Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months.
Shipping	The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature listed below.
Usage	For Research Use Only

Target Details

Target Function	Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. Can associate with lipoproteins and may play a role in lipid metabolism.
Subcellular Location	Secreted.
Database References	HGNC: 4888 OMIM: 134371 KEGG: hsa:3078 STRING: 9606.ENSP00000314299 UniGene: Hs.575869
Tissue Specificity	Expressed by the liver and secreted in plasma.

Gene Functions References

Novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein. This deletion was found in four family members presenting with a familial dominant glomerulopathy. PMID: 28729035
Plasma FHR-1 and the FHR-1/fH ratio were elevated in IgA nephropathy and associated with progressive disease. PMID: 28673452
A role of FHR-1 in IgA nephropathy pathogenesis is to compete with complement regulation by factor H. PMID: 28637589
We conclude that the relationship between complement-regulatory proteins CFHR1 and CFHR3 and response to anti-CD20 mAb therapy varies based on the specific anti-CD20 mAb used. PMID: 27528699
To our knowledge, this is the first evaluation of the involvement of the CFHR3/CFHR1 deletion and age-related macular degeneration in CFH Y402H polymorphism Brazilian patients. PMID: 26942649
These results identify C-reactive protein as a ligand for FHR-1 and suggest that FHR-1 enhances, rather than inhibits, complement activation, which may explain the protective effect of FHR-1 deficiency in age-related macular degeneration. PMID: 28533443
The CFHR1 level in plasma of T2DM patients were significantly higher than that of the healthy controls. PMID: 28604974
CFHL-1 is a CFH gene splice variant. PMID: 27814381
We investigated whether the causal sequence variant resides in the CFH gene or the neighboring complement factor H-related 1 (CFHR1) gene, and fine mapping Implicates a deletion of CFHR1 in protection from IgA nephropathy in Han Chinese PMID: 26940089
Next-generation sequencing of the CFH region identified putatively functional variants (missense, splice site and indel) on the four common haplotypes. We found no expression of any of the five CFH-related genes in the retina or RPE/Choroid/Sclera, in contrast to the liver, which is the main source of the circulating proteins. [CFHR1] PMID: 27196323
Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important implications for the role of these proteins in disease. PMID: 25979655
These results suggest that the combination of quantitative and qualitative variations in the complement proteins encoded by CFH, CFHR3 and CFHR1 genes is key for the association of these haplotypes with disease. PMID: 26163426
Genetic variants in CFH, CFHR3, and CFHR1 affect complement activation and thereby predispose patients to develop IgA nephropathy. PMID: 25205734
An average of 15.2% of factor H-autoantibody positive individuals with rheumatic diseases or hemolytic uremic syndrome had homozygous deficiency of CFHR1. PMID: 22894814
Data indicate that the autoantigenic epitope of complement factor H (CFH) and its homologous site in CFH-related protein-1(CFHR1) are structurally different PMID: 25659429
Prompt use of immunosuppressive agents and plasma exchanges are useful for improving outcomes in pediatric patients with anti-complement factor H-associated HUS. PMID: 24088957
Atypical haemolytic-uraemic syndrome due to heterozygous mutations of CFH/CFHR1-3 and complement factor H 479. PMID: 24333077
Factor H and the alternative spliced product FHL-1 are expressed in RPE cells. PMID: 24280833
we have assessed the relationship between GA and previously identified AMD-associated variants of genes (CFH, CFB, C3, FHR1, FRH3, and ARMS2/HTRA). PMID: 24557084
Results show non-coding CFH SNP (rs6677604) and the common deletion CNP147 were strongly correlated both with each other and with plasma CFH and CFHR1 concentrations in patients with age-related macular degeneration. PMID: 23873044
The CFHR1/complement factor H hybrid gene fusion protein contains the first three short consensus repeats of CFHR1 PMID: 23880784
These results provide evidence for a role of CFH and FHL-1 in cutaneous squamous cell carcinoma progression and identify them as progression markers and potential therapeutic targets. PMID: 23938460
Homozygous deletion in CFHR1 is strongly associated with occurrence of CFH antibodies in pediatric patients with atypical hemolytic uremic syndrome. PMID: 23243267
Identification and characterization of a unique CFHR1 mutation provides insights into the biology and pathogenic mechanisms underlying C3 glomerulopathy. PMID: 23728178
Lpd is a novel surface-exposed virulence factor of P. aeruginosa that binds Factor H, FHL-1, CFHR1, and plasminogen, and the Lpd-attached regulators are relevant for innate immune escape and most likely contribute to tissue invasion. PMID: 23071278
genetic variations in CFH and its related genes may contribute to hypertension risk in Chinese Hans PMID: 22848687
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. PMID: 22626820
we show that native factor H, factor H-like protein 1, and factor H-related protein 1 (CFHR1) bind to PTX3 PMID: 22786770
A significant association with deletion of CFHR1-4 was identified in patients who presented with bilateral geographic atrophy. PMID: 22558131
Reduced expression of the CFHR1 allele has been associated with higher risk to atypical Haemolytic Uraemic Syndrome in Spanish patients. PMID: 22136554
Analysis of the CFHR1 genotypes provide sufficient information to delineate the individual risk of developing age-related macular degeneration. PMID: 22247456
Data show that 698 CNPs loci overlap with known disease-associated or pharmacogenetic-related genes such as CFHR3, CFHR1, GSTTI and UGT2B17. PMID: 21677662
In this matched subset of Age-Related Eye Disease Study (AREDS) subjects, after adjusting for 2 known risk variants in CFH, CNP147 deletion statistically associates with diminished risk for AMD. PMID: 21856016
Anti-factor H autoantibodies in patients with autoimmune form of atypical hemolytic uremic syndrome crossreact with CFHR1. PMID: 21677636
Combined deletion of CFHR3 and CFHR1 is associated with a decreased risk of developing age-related macular degeneration. PMID: 21850184
A change in gene dosage of the encoded proteins CFHR3 and CFHR1 might account for the increased systemic lupus erythematosus. risk PMID: 21637784
deficiency of CFHR3 and CFHR1 results in a loss of complement control but enhances local regulation by factor H; alludes to critical balance between CFHR3, CFHR1 and factor H and emphasize role of complement regulation in age-related macular degeneration PMID: 20843825
Studies indicated that atypical HUS was linked with a complement alternative pathway dysregulation due to genetic defects but also to development of autoantibodies to factor H (FH). PMID: 20865640
Binding of the human complement regulators CFHR1 and factor H by streptococcal collagen-like protein 1 (Scl1) via their conserved C termini allows control of the complement cascade at multiple levels. PMID: 20855886
Data from haplotype analysis demonstrates the relationship between the CFH rs10737680 association and the CFHR1-3Delta association in age-related macular degeneration. PMID: 20581873
CFH and CFHR1, when bound on the surface of C. albicans, enhance antimicrobial activity of human neutrophils. PMID: 20008295
Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome. PMID: 19861685
Human complement regulatory factor H-like protein 1 (FHL-1) binds to Streptococcal pyogenes M18 surface protein Emm18. PMID: 15557185
the primary function of FHL-1 binding by T. denticola might be to facilitate adherence to FHL-1 present on anchorage-dependent cells and in the extracellular matrix PMID: 16239506
Deletion of two closely related genes, complement factor H-related 1 (CFHR1) and complement factor H-related 3 (CFHR3), increases the risk of atypical hemolytic uremic syndrome. PMID: 17367211
we have identified factor H-related protein 1 (FHR-1) as a novel protein that binds to Borrelia burgdorferi via CRASP-3, -4, and -5. PMID: 17538892
either lacked the CFHR1/CFHR3 completely (n = 14) or showed extremely low CFHR1/CFHR3 plasma levels (n = 2) are positive for factor H (CFH) autoantibodies PMID: 18006700
The binding of factor H and factor H-like protein 1 (FHL-1) from human sera to Aspergillus fumigatus conidia was shown by adsorption assays and immunostaining. PMID: 18039838
Deletion of CFHR1 and CFHR3 may account for a small portion of the protection from age-related macular degeneration associated with particular haplotypes in complement factor H. PMID: 18084039
CFH/CFHL1 binding site within borrelial BbCRASP-2 and identified single amino acid residues potentially involved in the interaction with both complement regulators PMID: 18824548

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Proteins are sensitive to heat, and freeze-drying can preserve the activity of the majority of proteins. It improves protein stability, extends storage time, and reduces shipping costs. However, freeze-drying can also lead to the loss of the active portion of the protein and cause aggregation and denaturation issues. Nonetheless, these adverse effects can be minimized by incorporating protective agents such as stabilizers, additives, and excipients, and by carefully controlling various lyophilization conditions.

Commonly used protectant include saccharides, polyols, polymers, surfactants, some proteins and amino acids etc. We usually add 8% (mass ratio by volume) of trehalose and mannitol as lyoprotectant. Trehalose can significantly prevent the alter of the protein secondary structure, the extension and aggregation of proteins during freeze-drying process; mannitol is also a universal applied protectant and fillers, which can reduce the aggregation of certain proteins after lyophilization.

Our protein products do not contain carrier protein or other additives (such as bovine serum albumin (BSA), human serum albumin (HSA) and sucrose, etc., and when lyophilized with the solution with the lowest salt content, they often cannot form A white grid structure, but a small amount of protein is deposited in the tube during the freeze-drying process, forming a thin or invisible transparent protein layer.

Reminder: Before opening the tube cap, we recommend that you quickly centrifuge for 20-30 seconds in a small centrifuge, so that the protein attached to the tube cap or the tube wall can be aggregated at the bottom of the tube. Our quality control procedures ensure that each tube contains the correct amount of protein, and although sometimes you can't see the protein powder, the amount of protein in the tube is still very precise.

To learn more about how to properly dissolve the lyophilized recombinant protein, please visit Lyophilization FAQs.