Product Name
PH / PAH, Polyclonal Antibody
Full Product Name
Anti-PH / PAH Antibody (aa351-400) IHC-plus
Product Synonym Names
Rabbit Polyclonal (IgG) to Human PH / PAH; Human PH / PAH; Phe-4-monooxygenase; PKU1; Phenylalanine 4-monooxygenase; Phe-4- monooxygenase; Phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU
Product Gene Name
anti-PH / 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
Mouse, Rat, Human
Specificity
PAH antibody detects endogenous levels of PAH.
Purity/Purification
Immunoaffinity purified
Form/Format
PBS, pH 7.4, 150 mM sodium chloride, 0.02% sodium azide, 50% glycerol.
Concentration
1 mg/ml (lot specific)
Immunogen
PH / PAH antibody was raised against synthetic peptide derived from human PAH (aa351-400).
Immunogen Description
Synthetic peptide derived from human PAH (aa351-400).
Immunogen Type
Synthetic peptide
Antigen Modification
aa351-400
Preparation and Storage
Store at -20 degree C.
Other Notes
Small volumes of anti-PH / 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-PH / PAH antibody
PAH Antibody, Phe-4-monooxygenase Antibody, PKU1 Antibody, PH Antibody, Phenylalanine 4-monooxygenase Antibody, Phe-4- monooxygenase Antibody, Phenylalanine hydroxylase Antibody, Phenylalanine-4-hydroxylase Antibody, PKU Antibody Description: 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.
Applications Tested/Suitable for anti-PH / PAH antibody
Immunohistochemistry (IHC) Paraffin, Western Blot (WB), ELISA (EIA)
Application Notes for anti-PH / PAH antibody
ELISA (1:10000)
IHC-P (10 ug/ml)
WB (1:500 - 1:1000)
Immunohistochemistry - Paraffin (IHC) of anti-PH / PAH antibody
Human Liver: Formalin-Fixed, Paraffin-Embedded (FFPE)
Immunohistochemistry - Paraffin (IHC) of anti-PH / PAH antibody
Human Kidney: Formalin-Fixed, Paraffin-Embedded (FFPE)
Western Blot (WB) of anti-PH / PAH antibody
Western blot analysis of lysate from HepG2 cells, using PAH antibody.
NCBI/Uniprot data below describe general gene information for PH / 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
UniProt Gene Name
PAH??[Similar Products]
UniProt Synonym Gene Names
PAH??[Similar Products]
UniProt Entry Name
PH4H_HUMAN
NCBI Summary for PH / 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 PH / 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: EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase
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 PH / PAH
1. Combining in silico analysis and molecular dynamics simulations (in total 3 mus) we described the structural impact of the mutations, which allowed us to separate 32 out of 34 mutations between groups A and B.
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