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Anti-CFI antibody produced in mouse
产品编号: | 3353110 |
规格: | purified immunoglobulin, buffered aqueous solution |
包装规格: | 50 μG |
产品类别: | 进口试剂 |
品牌: | Sigma-Aldrich |
优惠价: | 立即咨询 |
产品别名
Anti-CFI antibody produced in mouse
IF
FI
KAF
C3b-INA
基本信息
NACRES | NA.41 |
General description【一般描述】 | 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. (provided by RefSeq) |
Immunogen【免疫原】 | CFI (AAH20718.1, 1 a.a. ~ 377 a.a) full-length human protein. Sequence MKLLHVFLLFLCFHLRFCKVTYTSQEDLVEKKCLAKKYTHLSCDKVFCQPWQRCIEGTCVCKLPYQCPKNGTAVCATNRRSFPTYCQQKSLECLHPGTKFLNNGTCTAEGKFSVSLKHGNTDSEGIVEVKLVDQDKTMFICKSSWSMREANVACLDLGFQQGADTQRRFKLSDLSINSTECLHVHCRGLETSLAECTFTKRRTMGYQDFADVVCYTQKADSPMDDFFQCVNGKYISQMKACDGINDCGDQSDELCCKACQGKGFHCKSGVCIPSQYQCNGEVDCITGEDEVGCAGFASVAQEETEILTADMDAERRRIKSLLPKLSCGVKNRMHIRRKRIVGGKRAQLGKMKQISLDIFKGLHRVYAILEFCCIILK |
Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
biological source【生物来源】 | mouse |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | purified immunoglobulin |
antibody product type | primary antibodies |
clone【克隆】 | polyclonal |
form【形式】 | buffered aqueous solution |
mol wt【分子量】 | antigen ~42.4 kDa |
species reactivity | human |
technique(s) | western blot: 1 μg/mL |
NCBI accession no.【NCBI登记号】 | BC020718.1 |
UniProt accession no.【UniProt登记号】 | P05156 |
shipped in【运输】 | dry ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... CFI(3426) |
安全信息
Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
WGK | WGK 1 |