CFI (light chain) Blocking Peptide | CFI blocking peptide
CFI (light chain) Blocking Peptide (C-term)
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: EC 3.4.21.45; Secreted; Protease; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 4q25
Cellular Component: extracellular region; extracellular space
Molecular Function: protein binding; serine-type endopeptidase activity
Biological Process: regulation of complement activation
Disease: Complement Factor I Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 3; Macular Degeneration, Age-related, 13
Research Articles on CFI
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Product Notes
The CFI cfi (Catalog #AAA9230286) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "CFI (light chain), Blocking Peptide" 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
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