CFH Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| IHC-P, WB, IF, E |
---|---|
Primary Accession | P08603 |
Other Accession | NP_000177.2 |
Reactivity | Human |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 139096 Da |
Antigen Region | 751-780 aa |
Gene ID | 3075 |
---|---|
Other Names | Complement factor H, H factor 1, CFH, HF, HF1, HF2 |
Target/Specificity | This CFH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 751-780 amino acids of human CFH. |
Dilution | IF~~1:25 WB~~1:2000 IHC-P~~1:50~100 |
Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | CFH Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | CFH |
---|---|
Synonyms | HF, HF1, HF2 |
Function | Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces (PubMed:21285368, PubMed:25402769). Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of the complement amplification loop (PubMed:19503104, PubMed:26700768). As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b (PubMed:18252712, PubMed:23332154, PubMed:28671664). In addition, mediates several cellular responses through interaction with specific receptors. For example, interacts with CR3/ITGAM receptor and thereby mediates the adhesion of human neutrophils to different pathogens. In turn, these pathogens are phagocytosed and destroyed (PubMed:20008295, PubMed:9558116). |
Cellular Location | Secreted. |
Tissue Location | Expressed in the retinal pigment epithelium (at protein level) (PubMed:25136834). CFH is one of the most abundant complement components in blood where the liver is the major source of CFH protein in vivo. in addition, CFH is secreted by additional cell types including monocytes, fibroblasts, or endothelial cells (PubMed:2139673, PubMed:25136834, PubMed:2968404, PubMed:6444659) |
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Background
This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short concensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
References
Dieterich, R., et al. Infect. Immun. 78(11):4467-4476(2010)
Sofat, R., et al. Atherosclerosis 213(1):184-190(2010)
Davila, S., et al. Nat. Genet. 42(9):772-776(2010)
Scambi, C., et al. PLoS ONE 5 (8), E12162 (2010) :
Bunkenborg, J., et al. Proteomics 4(2):454-465(2004)
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