Sign in or Register   Sign in or Register
  |  

Mouse Anti-CFHR1 Recombinant Antibody (442127) (CBMAB-C1300-LY)

This product is antibody recognizes CFHR1. The antibody 442127 immunoassay techniques such as: WB, IP.
See all CFHR1 antibodies
Published Data
Fig.1 Western blot analysis of CFH, CFHR1, and CFHR3 expression in the UI/Mayo Lymphoma SPORE.
...View More

Summary

Host Animal
Mouse
Specificity
Human
Clone
442127
Antibody Isotype
IgG2b
Application
WB, IP

Basic Information

Immunogen
Mouse myeloma cell line NS0-derived recombinant human Complement Factor H-related 1/CFHR1, Glu19-Ala328, Accession # Q03591
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Complement Factor H Related 1
Introduction
This gene encodes a secreted protein belonging to the complement factor H protein family. It binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It is proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement, and facilitating tissue invasion. Mutations in this gene are associated with an increased risk of atypical hemolytic-uremic syndrome. [provided by RefSeq, Oct 2009]
Entrez Gene ID
3078
UniProt ID
Q03591
Alternative Names
Complement Factor H Related 1; Complement Factor H-Related 1 Pseudogene; H Factor (Complement)-Like 1; H Factor (Complement)-Like 2; H Factor-Like Protein 1; H-Factor-Like 1; CFHL1P; CFHR1P; CFHL1; FHR-1;
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.
Biological Process
Complement activation Source: ProtInc
Cytolysis by host of symbiont cells Source: UniProtKB
Negative regulation of protein binding Source: UniProtKB
Regulation of complement activation Source: Reactome
Cellular Location
Secreted
Involvement in disease
Hemolytic uremic syndrome atypical 1 (AHUS1): Disease susceptibility is associated with variants affecting the gene represented in this entry. A deletion encompassing CFHR1 and CFHR3 is associated with an increased risk of atypical hemolytic uremic syndrome, likely due to a defective regulation of complement activation (PubMed:17367211). Some patients carrying the deletion have serum anti-CFH autoantibodies (PubMed:18006700). 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.
PTM
N-glycosylated. Two forms are observed; one with a single side chain and the other with two.

Skerka, C., Pradel, G., Halder, L. D., Zipfel, P. F., Zipfel, S. L., & Strauß, O. (2021). Factor H‐related protein 1: a complement regulatory protein and guardian of necrotic‐type surfaces. British Journal of Pharmacology, 178(14), 2823-2831.

Irmscher, S., Zipfel, S. L., Halder, L. D., Ivanov, L., Gonzalez-Delgado, A., Waldeyer, C., ... & Skerka, C. (2021). Factor H-related protein 1 (FHR-1) is associated with atherosclerotic cardiovascular disease. Scientific reports, 11(1), 1-15.

van Beek, A. E., Pouw, R. B., Brouwer, M. C., van Mierlo, G., Geissler, J., Ooijevaar-de Heer, P., ... & Kuijpers, T. W. (2017). Factor H-related (FHR)-1 and FHR-2 form homo-and heterodimers, while FHR-5 circulates only as homodimer in human plasma. Frontiers in immunology, 8, 1328.

Medjeral-Thomas, N. R., Lomax-Browne, H. J., Beckwith, H., Willicombe, M., McLean, A. G., Brookes, P., ... & Pickering, M. C. (2017). Circulating complement factor H–related proteins 1 and 5 correlate with disease activity in IgA nephropathy. Kidney international, 92(4), 942-952.

Trojnár, E., Józsi, M., Uray, K., Csuka, D., Szilágyi, Á., Milosevic, D., ... & Prohászka, Z. (2017). Analysis of linear antibody epitopes on factor H and CFHR1 using sera of patients with autoimmune atypical hemolytic uremic syndrome. Frontiers in immunology, 8, 302.

Tortajada, A., Gutiérrez, E., de Jorge, E. G., Anter, J., Segarra, A., Espinosa, M., ... & de Córdoba, S. R. (2017). Elevated factor H–related protein 1 and factor H pathogenic variants decrease complement regulation in IgA nephropathy. Kidney international, 92(4), 953-963.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-CFHR1 Recombinant Antibody (442127)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare