Full Product Name
PAH Antibody
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, Rat
Concentration
1.0mg/ml (lot specific)
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.
Applications Tested/Suitable for anti-PAH antibody
Western Blot (WB), Immunohistochemistry (IHC)
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
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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
Protein Family
Phenylalanine-4-hydroxylase
UniProt Gene Name
PAH??[Similar Products]
UniProt Synonym Gene Names
PAH??[Similar Products]
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: 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
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.
Precautions
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