IF monoclonal antibody (M01), clone 1B3
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant IF.
Immunogen
IF (NP_000195, 19 a.a. ~ 118 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
KVTYTSQEDLVEKKCLAKKYTHLSCDKVFCQPWQRCIEGTCVCKLPYQCPKNGTAVCATNRRSFPTYCQQKSLECLHPGTKFLNNGTCTAEGKFSVSLKH
Host
Mouse
Reactivity
Human
Isotype
IgG2b Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.74 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Transfected lysate)
Western Blot analysis of CFI expression in transfected 293T cell line by IF monoclonal antibody (M01), clone 1B3.
Lane 1: CFI transfected lysate (Predicted MW: 42.4 KDa).
Lane 2: Non-transfected lysate.
Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged IF is approximately 0.3ng/ml as a capture antibody.ELISA
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Gene Info — CFI
Entrez GeneID
3426GeneBank Accession#
NM_000204Protein Accession#
NP_000195Gene Name
CFI
Gene Alias
C3B-INA, FI, IF, KAF
Gene Description
complement factor I
Omim ID
217030Gene Ontology
HyperlinkGene Summary
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
Other Designations
C3B/C4B inactivator|C3b-inactivator|I factor (complement)|Konglutinogen-activating factor|complement component I|complement control protein factor I|light chain of factor I
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Interactome
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Pathway
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Disease
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