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 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.

Specificity:

Recognizes endogenous levels of Fumarylacetoacetase protein.

Alternative names:

Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase

Species reactivity:

Human

Host:

Rabbit

Ig type:

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

Immunogen:

Recombinant full length protein of human Fumarylacetoacetase

Purification & Purity:

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

Molecular weight:

~45 kDa

Applications:

WB, IHC, IF/ICC

Usage:

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

Storage:

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

Precautions:

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

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