CFI Antibody - C-terminal region
Produktgrößen
25ul
£177,00
ARP71464-P050-25UL
100 ul
£412,00
ARP71464-P050-100UL
Über dieses Produkt
- SKU:
- ARP71464-P050
- Zusätzliche Namen:
- FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA
- Klonalität:
- Polyclonal
- Konzentration:
- 0.5 mg/ml
- 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 uraemic syndrome; a disease characterized by acute renal failure; microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
- Host:
- Rabbit
- Immunogen:
- The immunogen is a synthetic peptide directed towards the C-terminal region of Human CFI
- Molekulargewicht:
- 64kDa
- Aufreinigung:
- Affinity Purified
- Versandbedingungen:
- Blue Ice
- Lagerbedingungen:
- For short term use; store at 2-8C up to 1 week. For long term storage; store at -20C in small aliquots to prevent freeze-thaw cycles.
- Hersteller:
- Aviva Systems Biology
- Typ:
- Antibodies: Polyclonal Antibody
- Datenblatt des Herstellers:html_datasheet.php