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Antikörper

CFI Antibody

Produktgrößen
50ul
£264,00
OAAN01399-50UL
100ul
£379,00
OAAN01399-100UL
200ul
£581,00
OAAN01399-200UL
Über dieses Produkt
SKU:
OAAN01399
Zusätzliche Namen:
FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA
Klonalität:
Polyclonal
Konjugat:
Unconjugated
Weitere 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.
Gendetails:
complement factor I
Host:
Rabbit
Immunogen:
Recombinant protein of human CFI
Isotyp:
IgG
Molekulargewicht:
66 kDa
Proteindetails:
Complement factor I
Aufreinigung:
Affinity purified against immunogen
Versandbedingungen:
Blue Ice
Lagerbedingungen:
Store at -20C. Avoid repeated freeze/thaw cycles.
Hersteller:
Aviva Systems Biology
Typ:
Antibodies: Polyclonal Antibody