Full Product Name
FAH Antibody; FITC conjugated
Product Synonym Names
Fumarylacetoacetase; Beta-diketonase; Fumarylacetoacetate hydrolase; FAH
Product Gene Name
anti-FAH antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
MBS7003738 Technical Datasheet
Technical Datasheet PDF
3D Structure
ModBase 3D Structure for P16930
Purity/Purification
>95%,Protein G purified
Immunogen
Recombinant human Fumarylacetoacetase protein
Storage Buffer
Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-66223 / sc-67288
Preparation and Storage
Shipped at 4 degree C. Upon delivery, aliquot and store at -20 degree C or -80 degree C.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
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.
Related Product Information for
anti-FAH antibody
cytosol, extracellular exosome, fumarylacetoacetase activity, cellular nitrogen compound metabolic process, L-phenylalanine catabolic process, small molecule metabolic process, tyrosine catabolic process
Applications Tested/Suitable for anti-FAH antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for FAH. 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
UniProt Protein Name
Fumarylacetoacetase
UniProt Synonym Protein Names
Beta-diketonase; Fumarylacetoacetate hydrolase
Protein Family
Fumarylacetoacetase
UniProt Gene Name
FAH??[Similar Products]
UniProt Synonym Gene Names
FAA??[Similar Products]
UniProt Entry Name
FAAA_HUMAN
NCBI Summary for FAH
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 FAH
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: Amino Acid Metabolism - tyrosine; EC 3.7.1.2; Hydrolase
Chromosomal Location of Human Ortholog: 15q25.1
Cellular Component: cytosol
Molecular Function: fumarylacetoacetase activity; metal ion binding; protein binding
Biological Process: arginine catabolic process; L-phenylalanine catabolic process; tyrosine catabolic process
Disease: Tyrosinemia, Type I
Research Articles on FAH
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
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