Product Name :
FAH polyclonal antibody 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. Product :
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2 Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
FAH polyclonal antibody detects endogenous levels of FAH protein. Immunogen :
Recombinant full length Human FAH. Conjugate :
Unconjugated Modification :
Unmodification

• 
  WesternBlot (WB) analysis of FAH polyclonal antibody
• 
  WesternBlot (WB) analysis of FAH polyclonal antibody

Bioworld Biotech only provide peptides for our antibodies and do not provide additional peptide customization services.

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Price/Size :

USD 368/1mg/vial

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Tips: 

For phospho antibody, we provide phospho peptide（0.5mg) and non-phospho peptide(0.5mg).

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Describe :

Blocking peptides are peptides that bind specifically to the target antibody and block antibody binding. These peptide usually contains the epitope recognized by the antibody. Antibodies bound to the blocking peptide no longer bind to the epitope on the target protein. This mechanism is useful when non-specific binding is an issue, for example, in Western blotting (WB) and Immunohistochemistry (IHC). By comparing the staining from the blocked antibody versus the antibody alone, one can see which staining is specific; Specific binding will be absent from the western blot or IHC performed with the neutralized antibody.

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Formula:

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 10 mg/ml.The purity is >90%,tested by HPLC and MS.

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Storage:

The freeze-dried powder is more stable. For short time at 2-8°C. For long term storage store at -20°C. 

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Note :

This product is for research use only (RUO only). Not for use in diagnostic or therapeutic procedures.

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