Menselijke CFI-eiwit, zijn label

Description :Recombinant human CFI Protein with C-terminal6Ã-His tagBackground : 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. Molecular Characterization: mass of 64.3 kDa after removal of the signal peptide. The apparent molecular mass of CFI-His is approximately 70-100 kDa due to glycosylation.Tag :C-6×His tag

FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA

P05156

CFI

The purity of the protein is greater than 85% as determined by SDS-PAGE and Coomassie blue staining.

Lyophilized from sterile PBS, pH 7.4. Normally 5 % - 8% trehalose is added as protectants before lyophilization.

Store at -20°C to -80°C for 12 months in lyophilized form. After reconstitution, if not intended for use within a month, aliquot and store at -80°C (Avoid repeated freezing and thawing) . Lyophilized proteins are shipped at ambient temperature.