Factor I (CFI) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, IHC, WB
Recommend Dilution	ELISA. Western Blot: 1/200-1/2000. Immunohistochemistry: 1/50-1/500.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide derived from an internal domain of Human CFI
Specificity	Reacts with 65 kDa CFI protein.
Formulation	0.1M Tris, 0.1M Glycine, 2% Sucrose State: Purified State: Lyophilized purified powder Preservative: None
Reconstitution Method	Restore in distilled water.
Purification	Affinity Chromatography on Protein A
Conjugation	Unconjugated
Storage Condition	Store lyophilized at 2-8°C for 6 months or at -20°C long term. After reconstitution store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C long term. Avoid repeated freezing and thawing.
Gene Name	complement factor I
Database Link	Entrez Gene 3426 Human UniProt ID P05156
Background	Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3) [MIM:612923]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. 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.
Synonyms	CFI, IF, KAF, AHUS3, C3BINA, C3b-INA
Reference Data

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