CFI DNAxPab
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Specification
Product Description
Rabbit polyclonal antibody raised against a partial-length human CFI DNA using DNAx™ Immune technology.
Technology
Immunogen
Extracellular membrane domain (ECD) human DNA
Host
Rabbit
Reactivity
Human
Purification
Protein A
Quality Control Testing
Antibody reactive against mammalian transfected lysate.
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Transfected lysate)
Immunofluorescence (Transfected cell)
Flow Cytometry (Transfected cell)
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Gene Info — CFI
Entrez GeneID
3426GeneBank Accession#
BC020718.1Protein Accession#
AAH20718.1Gene Name
CFI
Gene Alias
C3B-INA, FI, IF, KAF
Gene Description
complement factor I
Omim ID
217030Gene Ontology
HyperlinkGene Summary
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. [provided by RefSeq
Other Designations
C3B/C4B inactivator|C3b-inactivator|I factor (complement)|Konglutinogen-activating factor|complement component I|complement control protein factor I|light chain of factor I
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Interactome
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Pathway
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Disease
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