Full Product Name
PAH Antibody
Product Synonym Names
PH; PKU; PKU1
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
Specificity
The antibody detects endogenous level of total PAH protein.
Purity/Purification
Antibodies were purified by affinity purification using immunogen.
Form/Format
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Concentration
1.0 mg/ml (lot specific)
Immunogen Type
Recombinant Protein
Immunogen Description
Recombinant protein of human PAH.
Preparation and Storage
Store at -20 degree C
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
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.
Product Categories/Family for anti-PAH antibody
Total protein Ab
Applications Tested/Suitable for anti-PAH antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-PAH antibody
Western blotting: 1:500 - 1:2000
Immunohistochemistry: 1:50 - 1:100
Western Blot (WB) of anti-PAH antibody
Western blot analysis of extracts of various cell lines, using PAH antibody.
Immunohistochemistry (IHC) of anti-PAH antibody
Immunohistochemical analysis of paraffin-embedded human esophageal cancer using PAH antibody at dilution of 1:100 (400x lens).
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: Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; EC 1.14.16.1; Oxidoreductase
Chromosomal Location of Human Ortholog: 12q22-q24.2
Cellular Component: cytosol
Molecular Function: amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity
Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process
Disease: Phenylketonuria
Research Articles on PAH
1. G are the most common mutations in PAH in phenylalanine hydroxylase deficiency patients.">R241C, R408Q and Ex6-96A>G are the most common mutations in PAH in phenylalanine hydroxylase deficiency patients.
Precautions
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Disclaimer
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