FAH Polyclonal Antibody-BS8270

FAH Polyclonal Antibody

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Sizes: 50µl, 100µl

Catalogue Numbers: BS8270-50, BS8270-100

Swiss-Prot: P16930

Host: Rabbit

FAH Polyclonal Antibody-BS8270

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Reactivity: Human, Mouse, Rat

Applications: WB, IHC

All Applications: WB: 1:500~1:2000
IHC: 1:50~1:200

Background: Fumarylacetoacetase is a 419 amino acid protein encoded by the human gene FAH. Fumarylacetoacetase catalyzes the hydrolysis of 4-fumarylacetoacetate, an intermediate in the metabolism of tyrosine, into acetoacetate and fumarate. Defects in FAH are the cause of tyrosinemia type I. It is an autosomal recessive inborn error of metabolism that occurs in both an acute and a chronic form. Clinical characteristics of the acute form include hepatic failure and death in infancy, whereas children with the chronic form have renal tubular dysfunction and hypophosphatemic rickets, progressive liver disease with development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Tyrosinemia type I is a rare condition, except in the Saguenay-lac-St-Jean region (province of Quebec, Canada) where the frequency is 1/1,846 newborns as the result of a founder effect.

Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Specificity: FAH polyclonal antibody detects endogenous levels of FAH protein.

Bioworld Molecular Weight: ~ 46 kDa

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: WesternBlot (WB) analysis of FAH polyclonal antibody, WesternBlot (WB) analysis of FAH polyclonal antibody

Alternative Name: Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase; FAH

Immunogen: Recombinant full length Human FAH.

Modification: Unmodified

Conjugate: Unconjugated