Product Name
PAH / PH, Recombinant Protein
Full Product Name
Recombinant Human PAH / PH Protein (415 Asn/Asp, His tag)
Product Synonym Names
PH, PKU, PKU1
Product Gene Name
PAH / PH recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P00439
Host
Baculovirus-Insect Cells
Purity/Purification
> 90 % as determined by SDS-PAGE
Form/Format
Lyophilized from sterile 20mM Tris, 500mM NaCl, pH 8.0, 10% glycerol
Endotoxin
< 1.0 EU per mug of the protein as determined by the LAL method
Preparation and Storage
Samples are stable for up to twelve months from date of receipt at -70 degree C
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
Small volumes of PAH / PH recombinant protein 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
PAH / PH recombinant protein
Background: PAH (phenylalanine hydroxylase), also known as PH, belongs to the biopterin-dependent aromatic amino acid hydroxylase family. It contains 1 ACT domain, N-terminal region of PAH is thought to contain allosteric s for phenylalanine and to constitute an "inhibitory" domain that regulates the activity of a catalytic domain in the C-terminal portion of the molecule. In humans, PAH is expressed both in the liver and the kidney, and there is some indication that it may be differentially regulated in these tissues. PAH catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. It is one of three members of the pterin-dependent amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin and a non-heme iron for catalysis. Defects in PAH are the cause of phenylketonuria (PKU). PKU is an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol.
Description: A DNA sequence encoding the human PAH (P00439) (Met 1-Lys 452) (415 Asn/Asp) was expressed, with a polyhistidine tag at the N-terminus.
Application Notes for PAH / PH recombinant protein
The recombinant human PAH consists of 471 amino acids and predicts a molecular mass of 54 kDa. It migrates as an approximately 50 KDa band in SDS-PAGE under reducing conditions.
SDS-PAGE of PAH / PH recombinant protein
NCBI/Uniprot data below describe general gene information for PAH / PH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000268.1
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NCBI GenBank Nucleotide #
NM_000277.1
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UniProt Primary Accession #
P00439
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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
UniProt Gene Name
PAH [Similar Products]
UniProt Synonym Gene Names
PAH [Similar Products]
NCBI Summary for PAH / PH
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 / PH
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: Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; EC 1.14.16.1; Oxidoreductase
Chromosomal Location of Human Ortholog: 12q23.2
Cellular Component: cytosol
Molecular Function: phenylalanine 4-monooxygenase activity
Biological Process: amino acid biosynthetic process; L-phenylalanine catabolic process
Disease: Phenylketonuria
Research Articles on PAH / PH
1. The results of the in vitro residual PAH activity have major implications, both for our understanding of genotype-phenotype correlations, and thereby existing inconsistencies, but also for the elucidation of the molecular basis of tetrahydrobiopterin (BH4) responsiveness.
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