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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
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
335311050 μG4610
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
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