Background：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.
仕様
Synonyms：AHUS3,ARMD13,C3b INA,C3b inactivator ,C3B/C4B inactivator,C3BINA,CFAI,Cfi,Complement component I,Complement control protein factor I,Complement factor I,Complement factor I heavy chain,Complement factor I light chain,F1,factor I ,FactorI,FI,I factor,IF,KAF,Konglutinogen activating factor,Light chain of factor I ,OTTHUMP00000219728,OTTHUMP00000221928
Host：Rabbit
Reactivity：Human,Mouse
Applications：WB,IF
Concentration：1mg/ml
Immunogen：Recombinant protein of human CFI
Purification Method：Affinity purification
Clonality：Polyclonal
Conjugation：Unconjugated
Buffer：Buffer： PBS with 0.02％ sodium azide, 50％ glycerol, pH7.3.
