|Application ||WB, E|
|Calculated MW||65750 Da|
|Other Names||Complement factor I, C3B/C4B inactivator, Complement factor I heavy chain, Complement factor I light chain, CFI, IF|
|Target/Specificity||IF (NP_000195, 19 a.a. ~ 118 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.|
|Format||Clear, colorless solution in phosphate buffered saline, pH 7.2 .|
|Storage||Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.|
|Precautions||IF Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.|
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Provided below are standard protocols that you may find useful for product applications.
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 uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome. Maga TK, et al. Hum Mutat, 2010 Jun. PMID 20513133.Genetic variants near TIMP3 and high-density lipoprotein-associated loci influence susceptibility to age-related macular degeneration. Chen W, et al. Proc Natl Acad Sci U S A, 2010 Apr 20. PMID 20385819.Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score. Rose JE, et al. Mol Med, 2010 Jul-Aug. PMID 20379614.A systematic gene-based screen of chr4q22-q32 identifies association of a novel susceptibility gene, DKK2, with the quantitative trait of alcohol dependence symptom counts. Kalsi G, et al. Hum Mol Genet, 2010 Jun 15. PMID 20332099.Genetic disorders in complement (regulating) genes in patients with atypical haemolytic uraemic syndrome (aHUS). Westra D, et al. Nephrol Dial Transplant, 2010 Jul. PMID 20106822.
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