CFI Rabbit Polyclonal Antibody (CAB5623)
- SKU:
- CAB5623
- Product Type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Immunology
Description
Product Name: | CFI Rabbit Polyclonal Antibody |
SKU: | CAB5623 |
Size: | 20uL, 100uL |
Isotype: | IgG |
Host Species: | Rabbit |
Reactivity: | Human,Mouse |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 19-300 of human CFI (NP_000195.2). |
Sequence: | KVTY TSQE DLVE KKCL AKKY THLS CDKV FCQP WQRC IEGT CVCK LPYQ CPKN GTAV CATN RRSF PTYC QQKS LECL HPGT KFLN NGTC TAEG KFSV SLKH GNTD SEGI VEVK LVDQ DKTM FICK SSWS MREA NVAC LDLG FQQG ADTQ RRFK LSDL SINS TECL HVHC RGLE TSLA ECTF TKRR TMGY QDFA DVVC YTQK ADSP MDDF FQCV NGKY ISQM KACD GIND CGDQ SDEL CCKA CQGK GFHC KSGV CIPS QYQC NGEV DCIT GEDE VGCA GFAS VA |
Tested Applications: | WB ELISA |
Recommended Dilution: | WB,1:500 - 1:2000 |
Synonyms: | FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA; CFI |
Positive Sample: | Human serum |
Conjugate: | Unconjugated |
Cellular Localization: | Secreted, extracellular space. |
Calculated MW: | 66kDa |
Observed MW: | 60kDa |
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.
Purification Method: | Affinity purification |
Gene ID: | 3426 |
Storage Buffer: | Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |