catalog number :
MBS9200125
products full name :
PAH Antibody (Center)
products short name :
[PAH]
products name syn :
[Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase; PAH]
other names :
[phenylalanine-4-hydroxylase; Phenylalanine-4-hydroxylase; phenylalanine-4-hydroxylase; phenylalanine hydroxylase; Phe-4-monooxygenase]
products gene name :
[PAH]
other gene names :
[PAH; PAH; PH; PKU; PKU1; PAH]
uniprot entry name :
PH4H_HUMAN
reactivity :
Human, mouse, rat (Predicted Reactivity: Bovine)
specificity :
This PAH antibody is generated from a mouse immunized with a KLH conjugated synthetic peptide between 127-161 amino acids from the Central region of human PAH.
purity :
Purified Mouse Monoclonal Antibody (Mab)
form :
Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.
storage stability :
Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
tested application :
Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)
app notes :
IHC~~1:25. WB~~1:1000
image1 heading :
Immunohistochemistry (IHC)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunohistochemistry (IHC)
image4 heading :
Western Blot (WB)
image4 description :
Western blot analysis of lysates from HepG2 cell line and mouse liver rat liver tissue lysates (from left to right) using PAH Antibody (Center). MBS9200125 was diluted at 1:1000 at each lane. A goat anti-mouse IgG H&L(HRP) at 1:3000 dilution was used as the secondary antibody. Lysates at 35ug per lane.
other info1 :
Calculated MW: 51862
products categories :
Crown Antibodies; Metabolism; Signal Transduction
products references :
Kwok S.C.M.,et al.Biochemistry 24:556-561(1985). Scriver C.R.,et al.Submitted (SEP-1997) to the EMBL/GenBank/DDBJ databases. Cotton R.G.,et al.Biochem. J. 255:193-196(1988). Miranda F.F.,et al.J. Biol. Chem. 277:40937-40943(2002). Siltberg-Liberles J.,et al.Gene 427:86-92(2008).
ncbi acc num :
NP_000268.1
ncbi gb acc num :
NM_000277.1
ncbi pathways :
Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053); Fatty Acid Biosynthesis Pathway (5053)
ncbi summary :
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 summary :
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; Oxidoreductase; EC 1.14.16.1. 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