Factor I antibody
Product Sizes
100 ul
GTX32531-100UL
About this Product
- SKU:
- GTX32531
- Additional Names:
- complement factor I , AHUS3 , ARMD13 , C3BINA , C3b-INA , FI , IF , KAF
- Application:
- IHC-Paraffin, Western Blot
- Buffer:
- PBS, 50% Glycerol, 0.02% Sodium azide.
- Clonality:
- Polyclonal
- 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]
- Host:
- Rabbit
- Immunogen:
- Recombinant fusion protein containing a sequence corresponding to amino acids 19-300 of human CFI (NP_000195.2).
- Isotype:
- IgG
- Physical State:
- Liquid
- Purification:
- Affinity Purified
- Reactivities:
- Human
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
- Supplier:
- GeneTex
- Type:
- Antibody: Polyclonal Antibody
- Manufacturer's Data Sheet:Download
