Human CFI Protein; His Tag

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£174.00

SKU:: PME101396
Additional Names:: FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA
Molecular Weight:: The protein has a predicted molecular 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.
Purity:: The purity of the protein is greater than 85% as determined by SDS-PAGE and Coomassie blue staining.
Storage Conditions:: Store at -20[o]C to -80[o]C for 12 months in lyophilized form. After reconstitution; if not intended for use within a month; aliquot and store at -80[o]C (Avoid repeated freezing and thawing).Lyophilized proteins are shipped at ambient temperature.
Supplier:: DIMA Biotechnology Ltd
Formulation:: Lyophilized from sterile PBS; pH 7.4. Normally 5 % - 8% trehalose is added as protectants before lyophilization. Please see Certificate of Analysis for specific instructions.
Target:: CFI
CFI(Lys19-Val583) 6×His tag:: CFI(Lys19-Val583) 6×His tag
Extra Details:: 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. [provided by RefSeq; Dec 2015]

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