Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase; FAH

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This FAH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 7-33 amino acids from the N-terminal region of human FAH.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.25 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-FAH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes the last enzyme in the tyrosine
catabolism pathway. FAH deficiency is associated with Type 1
hereditary tyrosinemia (HT).

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB~~1:1000

FAH Antibody (N-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the FAH antibody detected the FAH protein (arrow).

FAH Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of FAH Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.

Confocal immunofluorescent analysis of FAH Antibody (N-term) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).

46374

fumarylacetoacetate hydrolase (fumarylacetoacetase)

fumarylacetoacetase

Fumarylacetoacetase

FAA??[Similar Products]

FAAA_HUMAN

This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008]

FAH: Defects in FAH are the cause of tyrosinemia type 1 (TYRO1). An inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include hepatic necrosis, renal tubular injury, episodic weakness, self-mutilation, and seizures. Renal tubular dysfunction is associated with phosphate loss and hypophosphataemic rickets. Progressive liver disease can lead to the 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. Belongs to the FAH family.

Protein type: EC 3.7.1.2; Amino Acid Metabolism - tyrosine; Hydrolase

Chromosomal Location of Human Ortholog: 15q25.1

Cellular Component: cytosol

Molecular Function: fumarylacetoacetase activity; metal ion binding

Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process; arginine catabolic process

Disease: Tyrosinemia, Type I

Joslyn, G., et al. Alcohol. Clin. Exp. Res. 34(5):800-812(2010)
Liu, J., et al. Hum Brain Mapp 30(1):241-255(2009)
Ferreira, M.A., et al. Nat. Genet. 40(9):1056-1058(2008)
Bliksrud, Y.T., et al. J. Mol. Med. 83(5):406-410(2005)
Dreumont, N., et al. BMC Mol. Biol. 6 (1), 1 (2005) :

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.