Factor I (CFI) Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI2B5]

Specifications

Product Data
Clone Name	OTI2B5
Applications	IHC, WB
Recommend Dilution	WB 1:500
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Human recombinant protein fragment corresponding to amino acids 340-583 of human CFI(NP_000195) produced in E.coli.
Formulation	PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Concentration	0.5 mg/ml
Purification	Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
Conjugation	Biotin
Storage Condition	Store at -20°C as received.
Predicted Protein Size	63.4 kDa
Gene Name	complement factor I
Database Link	NP_000195 Entrez Gene 3426 Human UniProt ID P05156
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. [provided by RefSeq, Jul 2008]
Synonyms	AHUS3; ARMD13; C3b-INA; C3BINA; FI; IF; KAF
Reference Data
Protein Families	Druggable Genome, Protease, Secreted Protein
Protein Pathways	Complement and coagulation cascades

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