Rabbit anti-Human CFI Polyclonal Antibody | anti-CFI antibody
CFI (Light Chain) (C-Term) Rabbit pAb
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015]
Uniprot Description
CFI: Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3). 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. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency). CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections. Belongs to the peptidase S1 family.
Protein type: Protease; Secreted, signal peptide; EC 3.4.21.45; Secreted
Chromosomal Location of Human Ortholog: 4q25
Cellular Component: extracellular space; membrane; extracellular region; nucleus
Molecular Function: serine-type endopeptidase activity; metal ion binding; scavenger receptor activity
Biological Process: receptor-mediated endocytosis; regulation of complement activation; innate immune response; proteolysis; complement activation, classical pathway
Disease: Macular Degeneration, Age-related, 13; Complement Factor I Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 3
Research Articles on CFI
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Product Notes
The CFI cfi (Catalog #AAA8557569) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The CFI (Light Chain) (C-Term) Rabbit pAb reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CFI can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:2000. Researchers should empirically determine the suitability of the CFI cfi for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "CFI, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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