CFI Rabbit pAb
Sizes: 20μL, 100μL
Catalogue Numbers: A5623-20, A5623-100
Citations, Manuals and MSDS Available upon request.
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.
Category: Polyclonal Antibodies
Applications: WB, ELISA
Cross-reactivity: Human, Mouse
Protein Weight: 66kDa
Observed Molecular Weight: 60kDa
Immunogen: Recombinant protein (or fragment) .
Species: Human
GeneID: 3426
SWISS: P05156
Alternate Names: FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA; CFI
Source: Rabbit
Isotype: IgG
Purity: Affinity purification
Storage: Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS containing 50% glycerol, preserved with proclin300 or sodium azide (as specified on the Certificate of Analysis), pH 7.3.
Recommended Dilutions: WB, 1:500 - 1:2000 ELISA, Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Research Areas: Immunology Inflammation, Cell Intrinsic Innate Immunity Signaling Pathway.
NCBI Alias: CFI
Research Use Only