Key features and details
- Rabbit polyclonal to Fumarylacetoacetate hydrolase/FAA
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Fumarylacetoacetate hydrolase/FAA antibody
See all Fumarylacetoacetate hydrolase/FAA primary antibodies
DescriptionRabbit polyclonal to Fumarylacetoacetate hydrolase/FAA
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Horse, Cow, Pig
Recombinant fragment corresponding to Human Fumarylacetoacetate hydrolase/FAA aa 236-408.
ARDIQKWEYVPLGPFLGKSFGTTVSPWVVPMDALMPFAVPNPKQDPRPLP YLCHDEPYTFDINLSVNLKGEGMSQAATICKSNFKYMYWTMLQQLTHHSV NGCNLRPGDLLASGTISGPEPENFGSMLELSWKGTKPIDLGNGQTRKFLL DGDEVIITGYCQGDGYRIGFGQC
- Human fetal liver and fetal kidney lysates; Human fetal liver tissue
Previously labelled as Fumarylacetoacetate hydrolase.
FormLyophilized:Reconstitute in 200 µl sterile water.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 1% BSA, 98% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab140167 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/2000. Predicted molecular weight: 46 kDa.|
|IHC-P||1/100 - 1/500.|
RelevanceFumarylacetoacetate hydrolase / FAA 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.
- Beta diketonase antibody
- FAA antibody
- FAH antibody
All lanes : Anti-Fumarylacetoacetate hydrolase/FAA antibody (ab140167) at 1/1000 dilution
Lane 1 : Human fetal liver lysate
Lane 2 : Human fetal kidney lysate
Predicted band size: 46 kDa
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human fetal liver tissue labelling Fumarylacetoacetate hydrolase/FAA with ab140167 at 1/100 dilution.
ab140167 has been referenced in 2 publications.
- Chen J et al. Functional Comparison of IFN-a Subtypes Reveals Potent HBV Suppression by a Concerted Action of IFN-a and -? Signaling. Hepatology N/A:N/A (2020). PubMed: 32333814
- Li L et al. Fumarylacetoacetate Hydrolase Knock-out Rabbit Model for Hereditary Tyrosinemia Type 1. J Biol Chem 292:4755-4763 (2017). PubMed: 28053091