BD-PT1652

Factor I Rabbit Polyclonal Antibody

CFI; IF; Complement factor I; C3B/C4B inactivator

常规抗体

CFI

Complement factor I

WB

Human,Rat,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

3426

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=3426

P05156

http://www.uniprot.org/uniprotkb/P05156/entry

Q61129

http://www.uniprot.org/uniprot/Q61129

The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490

Factor I Polyclonal Antibody detects endogenous levels of Factor I protein.

WB 1:500-2000 IHC 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

Full lenth:66kD, heavy chain: 50-58kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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. [provided by Ref

Expressed in the liver by hepatocytes (PubMed:6327681). Also present in other cells such as monocytes, fibroblasts or keratinocytes (PubMed:6444659, PubMed:17320177).

Secreted, extracellular space. Secreted .

Complement and coagulation cascades;

catalytic activity:Inactivates complement subcomponents C3b, iC3b and C4b by proteolytic cleavage.,disease:Defects in CFI are the cause of complement factor I deficiency (CFI deficiency) [MIM:610984]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.,disease:Defects in CFI are the cause of component I deficiency (CFI deficiency) [MIM:217030]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.,disease:Defects in CFI may be associated with or predispose to hemolytic uraemic syndrome (HUS) [MIM:235400]. HUS, the most frequent cause of acute renal failure in childhood, is characterized by the association of acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. The majority of HUS cases occur after an episode of infectious diarrhea, and are associated with E.coli O157:H7 infection. However, atypical cases of HUS occur in the absence of infectious diarrhea, although less commonly. Some are inherited in either an autosomal dominant or a recessive pattern and these patients often experience relapse and progress to hypertension and chronic renal disease. Sporadic forms can occur with many of the same signs and symptoms.,function:Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.,online information:CFI mutation db,similarity:Belongs to the peptidase S1 family.,similarity:Contains 1 peptidase S1 domain.,similarity:Contains 1 SRCR domain.,similarity:Contains 2 LDL-receptor class A domains.,subunit:Heterodimer of a light and heavy chains linked by disulfide bonds.,tissue specificity:Plasma.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.