Full Product Name
PAH, ID (PAH, Phenylalanine-4-hydroxylase, Phe-4-monooxygenase)
Product Synonym Names
Anti -PAH, ID (PAH, Phenylalanine-4-hydroxylase, Phe-4-monooxygenase)
Product Gene Name
anti-PAH antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 12; NC_000012.11 (103232104..103311381, complement). Location: 12q22-q24.2
3D Structure
ModBase 3D Structure for P00439
Species Reactivity
Human, Mouse
Purity/Purification
Affinity Purified
Purified by Protein A affinity chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Immunogen
PAH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between amino acid 140-170 from the Central region of human PAH.
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
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
Antibodies; Abs to Enzymes
Applications Tested/Suitable for anti-PAH antibody
ELISA (EL/EIA), Western Blot (WB)
Application Notes for anti-PAH antibody
Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
NCBI/Uniprot data below describe general gene information for PAH. It may not necessarily be applicable to this product.
NCBI Accession #
AAA80910.1
[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[Similar Products]
NCBI Official Full Name
PAH
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; phe-4-monooxygenase; phenylalanine 4-monooxygenase
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. A, p.R261Q, p.R158Q and IVS2 + 5G>C.">The five most prevalent PAH mutations found in patients were p.R408W, IVS12 + 1G>A, p.R261Q, p.R158Q and IVS2 + 5G>C.
Precautions
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Disclaimer
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