Full Product Name
PAH Antibody; FITC conjugated
Product Synonym Names
Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase; PAH
Product Gene Name
anti-PAH antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P00439
Species Reactivity
Human, mouse
Purity/Purification
>95%,Protein G purified
Immunogen
Recombinant human Phenylalanine-4-hydroxylase 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-15109 / sc-15112
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-PAH 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-PAH antibody
cytosol, extracellular exosome, phenylalanine 4-monooxygenase activity, catecholamine biosynthetic process, cellular amino acid biosynthetic process, cellular nitrogen compound metabolic process, L-phenylalanine catabolic process, neurotransmitter biosynthetic process, small molecule metabolic process
Applications Tested/Suitable for anti-PAH antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for PAH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000268.1
[Other Products]
NCBI GenBank Nucleotide #
NM_000277.1
[Other Products]
UniProt Primary Accession #
P00439
[Other Products]
UniProt Secondary Accession #
Q16717; Q8TC14[Other Products]
UniProt Related Accession #
P00439[Other Products]
Molecular Weight
51,862 Da
NCBI Official Full Name
phenylalanine-4-hydroxylase
NCBI Official Synonym Full Names
phenylalanine hydroxylase
NCBI Official Symbol
PAH [Similar Products]
NCBI Official Synonym Symbols
PH; PKU; PKU1
[Similar Products]
NCBI Protein Information
phenylalanine-4-hydroxylase
UniProt Protein Name
Phenylalanine-4-hydroxylase
UniProt Synonym Protein Names
Phe-4-monooxygenase
Protein Family
Phenylalanine-4-hydroxylase
UniProt Gene Name
PAH [Similar Products]
UniProt Synonym Gene Names
PAH [Similar Products]
UniProt Entry Name
PH4H_HUMAN
NCBI Summary for PAH
PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Jul 2008]
UniProt Comments for PAH
PAH: phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.
Protein type: Oxidoreductase; EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis
Chromosomal Location of Human Ortholog: 12q22-q24.2
Cellular Component: cytosol
Molecular Function: amino acid binding; cofactor binding; iron ion binding; phenylalanine 4-monooxygenase activity; protein homodimerization activity
Biological Process: amino acid biosynthetic process; catecholamine biosynthetic process; L-phenylalanine catabolic process; neurotransmitter biosynthetic process; protein amino acid hydroxylation; tetrahydrobiopterin metabolic process; tyrosine biosynthetic process
Disease: Phenylketonuria
Research Articles on PAH
1. mutation spectrum of the gene PAH in the Qinghai population was similar to that in other populations in North China
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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