Factor I antibody [3R/8]
Product Sizes
100 ug
GTX41627-100UG
About this Product
- SKU:
- GTX41627
- Additional Names:
- complement factor I , AHUS3 , ARMD13 , C3BINA , C3b-INA , FI , IF , KAF
- Application:
- Block/Inhibit/Neutralise, ELISA, IHC-Frozen, Western Blot
- Buffer:
- Borate buffered saline, 0.1% Sodium azide.
- translate.label.attr.clone:
- 3R/8
- Clonality:
- Monoclonal
- Concentration:
- 1 mg/ml
- 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:
- Mouse
- Immunogen:
- Native human factor I from serum.
- Isotype:
- IgG1
- Physical State:
- Liquid
- Purification:
- Protein A Purified
- Reactivities:
- Human
- Shipping Conditions:
- Blue Ice
- Specificity:
- This antibody recognizes complement factor I. Clone 3R/8 blocks function of complement factor I.
- 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: Monoclonal Antibody
- Manufacturer's Data Sheet:Download
