Factor I is 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.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry and Immunocytochemistry.
Other applications not tested.
Recommended Dilutions:Western Blot: 0.5-2ug/mlImmunohistochemistry (Paraffin): 5-20ug/ml Immunocytochemistry: 5-20ug/ml Optimal dilutions to be determined by the researcher.
Included Protein Molecular Weight Marker:168631: Unstained Protein Molecular Weight Marker, 10-70kDSuppled as a liquid in 62.5mM Tris-H3PO4, pH 7.5, 1mM EDTA, 2% SDS, 100mM DTT, 1mM sodium azide, 0.01% bromo-phenol blue, 33% glycerol.
Ready to use; no need to heat, dilute or add reducing agents before use
Protein Bands: 10kD, 14kD, 18kD, 22kD, 26kD, 33kD, 44kD and 70kD
Double Intesity Bands: The 26kD, 18kD and 10kD bands are at double intensity to make location and size approximation of proteins easier
Usage: Allow the marker to reach room temperature and mix thoroughly before use to ensure that any solids that may have precipitated at -20°C will go back into solution.
Do not boil! Load the following volumes on SDS-PAGE gel:Mini gels: 3-5ul/wellLarge gel: 7ul/wellThe loading volumes listed above are recommended for gels with thickness of 0.75-1mm.
The loading volume should be doubled for 1.5mm thick gels
Store at -20°C Stable for 6 months after receipt.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A and peptide affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.4, 0.05% Proclin-300, 50% glycerol.
Specificity:Recognizes human CFI.
Isotype:IgG
Calc Applications Abbrev:E IC IHC WB
Calc Crossreactivity:Hu
Immunogen:Recombinant protein corresponding to Leu28-Gly243 of human CFI, expressed in E. coli.