Full Product Name
Anti-FAA Antibody
Product Gene Name
anti-FAA antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P16930
Specificity
Recombinant protein corresponding to full length FAA.
Purity/Purification
Affinity purified
Concentration
100ug/100ul (lot specific)
Raised In
Purified mouse monoclonal antibody generated using E.coli expressed recombinant protein FAA as immunogen.
Storage Buffer
PBS, pH 7.4 with 0.02% sodium azide.
Immunogen
Recombinant protein corresponding to full length PRS-III.
Preparation and Storage
This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.
Other Notes
Small volumes of anti-FAA 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.
Applications Tested/Suitable for anti-FAA antibody
ELISA (EIA), Western Blot (WB-Ce)
Application Notes for anti-FAA antibody
WB: 1:500, analysis of FAA expression in HEK293 whole cell lysate with Anti-FAA
Testing Data of anti-FAA antibody
NCBI/Uniprot data below describe general gene information for FAA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000128.1
[Other Products]
NCBI GenBank Nucleotide #
NM_000137.2
[Other Products]
UniProt Primary Accession #
P16930
[Other Products]
UniProt Secondary Accession #
Q53XA7; B2R9X1; D3DW95[Other Products]
UniProt Related Accession #
P16930[Other Products]
Molecular Weight
38,614 Da
NCBI Official Full Name
fumarylacetoacetase
NCBI Official Synonym Full Names
fumarylacetoacetate hydrolase (fumarylacetoacetase)
NCBI Official Symbol
FAH??[Similar Products]
NCBI Protein Information
fumarylacetoacetase; FAA; beta-diketonase
UniProt Protein Name
Fumarylacetoacetase
UniProt Synonym Protein Names
Beta-diketonase; Fumarylacetoacetate hydrolase
UniProt Gene Name
FAH??[Similar Products]
UniProt Entry Name
FAAA_HUMAN
NCBI Summary for FAA
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]
UniProt Comments for FAA
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
Product References and Citations for anti-FAA antibody
None
Research Articles on FAA
1. Four splicing mutations affecting exonic or intronic nucleotides of the FAH gene were identified in two hereditary tyrosinemia type I patients.
Precautions
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.
Disclaimer
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.
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