Product Description

Fumarylacetoacetase Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS67322-50, BS67322-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: Fumarylacetoacetate hydrolase is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). This 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 tubμlar dysfunction and hypophosphatemic rickets, progressive liver disease with development of hepatocellμlar carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment.

Category: Primary Antibody

Reactivity: Human

Host: Rabbit

Applications: WB, IHC, IF/ICC

Alternate Names: Fumarylacetoacetase, FAA, Beta-diketonase, Fumarylacetoacetate hydrolase

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: Recombinant fμll length protein of human Fumarylacetoacetase

Dilution: WB (1/500 - 1/2000), IHC (1/50 - 1/200), IF/ICC (1/10 - 1/100)

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

Specificity: Recognizes endogenous levels of Fumarylacetoacetase protein.

Molecular Weight: ~45 kDa

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

SwissProt: P16930

Product: Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Research Use Only