- Catalog Number: H00003426-B01
- Unit Size: 0.05 ml
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| Gene ID | 3426 | Reference Sequence | |
| Species Summary | Hu(+) | Application Summary | ELISA, WB |
| Purity | Host | Mouse |
| Storage | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| Background | 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. |
| Related Diseases | complement factor I deficiency, atypical hemolytic uraemic syndrome |
| Immunogen | IF (-, 1 a.a. ~ 377 a.a) full-length human protein. |
| Epitope | MKLLHVFLLFLCFHLRFCKVTYTSQEDLVEKKCLAKKYTHLSCDKVFCQPWQRCIEGTCVCKLPYQCPKNGTAVCATNRRSFPTYCQQKSLECLHPGTKFLNNGTCTAEGKFSVSLKHGNTDSEGIVEVKLVDQDKTMFICKSSWSMREANVACLDLGFQQGADTQRRFKLSDLSINSTECLHVHCRGLETSLAECTFTKRRTMGYQDFADVVCYTQKADSPMDDFFQCVNGKYISQMKACDGINDCGDQSDELC |
| Species Reactivity | Human. |
| Uses | Antibody Reactive Against Tissue Lysate and Transfected Lysate on Western Blot. It has also been used for ELISA. |
| Dilutions |
Western Blot 1:500, ELISA |
| Packaging | 0.05 ml of mouse antisera with 50% glycerol. |
| Buffer | This antibody is in antisera and the concentration is unavailable. |
| Preservative | No Preservative |
| Alternate Names | anti-FI antibody, anti-factor I antibody |
| Notes |
This antibody is supplied by Abnova. The immunizing protein may also available for this product. Unit cost for the protein is $ 250 (2 ug), $ 305 (10 ug), and $ 445 (25 ug). MaxPab is a registered trademark of Abnova. |
| Gene Symbol | CFI |
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