BT LAB

Artikelnummer: BT-AP03122-50ul

Factor I Polyclonal Antibody, 50ul

CFI 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.

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Product Specification

Basic Information

Brands:BT LAB

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Place of Origin:China

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Concentration:1 mg/ml

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Storage:-20°C for 1 year

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Application:WB, ELISA

Detection Target:CFI

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Research Areas:Immunology

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Clonality:Polyclonal

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Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

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