Overview

  • Product name

    Anti-Fumarylacetoacetate hydrolase/FAA antibody
    See all Fumarylacetoacetate hydrolase/FAA primary antibodies
  • Description

    Rabbit polyclonal to Fumarylacetoacetate hydrolase/FAA
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Horse, Cow, Pig
  • Immunogen

    Recombinant fragment corresponding to Human Fumarylacetoacetate hydrolase/FAA aa 236-408.
    Sequence:

    ARDIQKWEYVPLGPFLGKSFGTTVSPWVVPMDALMPFAVPNPKQDPRPLP YLCHDEPYTFDINLSVNLKGEGMSQAATICKSNFKYMYWTMLQQLTHHSV NGCNLRPGDLLASGTISGPEPENFGSMLELSWKGTKPIDLGNGQTRKFLL DGDEVIITGYCQGDGYRIGFGQC

  • Positive control

    • Human fetal liver and fetal kidney lysates; Human fetal liver tissue
  • General notes

    Previously labelled as Fumarylacetoacetate hydrolase. 

Properties

Applications

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.

Application Abreviews Notes
WB 1/1000 - 1/2000. Predicted molecular weight: 46 kDa.
IHC-P 1/100 - 1/500.

Target

  • Relevance

    Fumarylacetoacetate 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.
  • Database links

  • Alternative names

    • Beta diketonase antibody
    • FAA antibody
    • FAH antibody
    • Fumarylacetoacetase antibody
    • Fumarylacetoacetate antibody
    • Fumarylacetoacetate hydrolase antibody
    see all

Images

  • 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.

References

This product has been referenced in:

  • Li L  et al. Fumarylacetoacetate Hydrolase Knock-out Rabbit Model for Hereditary Tyrosinemia Type 1. J Biol Chem 292:4755-4763 (2017). Read more (PubMed: 28053091) »
See 1 Publication for this product

Customer reviews and Q&As

Application
Immunohistochemistry (Frozen sections)
Sample
Human Tissue sections (Liver)
Permeabilization
No
Specification
Liver
Blocking step
Serum as blocking agent for 30 minute(s) · Concentration: 10% · Temperature: 25°C
Fixative
Paraformaldehyde

Abcam user community

Verified customer

Submitted Feb 21 2018

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up