catalog number :
MBS2004517
products full name :
Polyclonal Antibody to Phenylalanine Hydroxylase (PAH)
products short name :
[Phenylalanine Hydroxylase (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]
purity :
Antigen-specific affinity chromatography followed by Protein A affinity chromatography
form :
Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.
storage stability :
Storage: Avoid repeated freeze/thaw cycles. Store at 4ºC for frequent use. Aliquot and store at -20ºC for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunoprecipitation (IP).
app notes :
Western blotting: 0.5-2 ug/mL;1:500-2000. Immunohistochemistry: 5-20 ug/mL;1:50-200. Immunocytochemistry: 5-20 ug/mL;1:50-200. Optimal working dilutions must be determined by end user.
image1 heading :
Western Blot (WB)
image2 heading :
Western Blot (WB)
image3 heading :
Immunohistochemistry (IHC)
image4 heading :
Immunohistochemistry (IHC)
image4 description :
DAB staining on IHC-P; Samples: Human Kidney Tissue.
image5 heading :
Knockout Validation
image5 description :
Knockout Validation: Lane 1: Wild-type Hela cell lysate; Lane 2: PAH knockout Hela cell lysate; Predicted MW: 51kd Observed MW: 55kd Primary Ab: 2ug/ml Rabbit Anti-Human PAH Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047)
other info1 :
Organism Species: Homo sapiens (Human). Source: Polyclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant PAH (Met1~Gln226) expressed in E.coli
other info2 :
Conjugated Antibody: The APC conjugated antibody version of this item is also available as catalog #MBS2067989
products categories :
Knock-Out Validation Antibody
ncbi acc num :
NP_000268.1
ncbi gb acc num :
NM_000277.2
ncbi pathways :
Abnormal Metabolism In Phenylketonuria Pathway (645287); Biogenic Amine Synthesis Pathway (198793); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Disease Pathway (530764); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Phenylalanine And Tyrosine Catabolism Pathway (106189); Phenylalanine Metabolism Pathway (82960)
ncbi summary :
This gene encodes a member of the biopterin-dependent aromatic amino acid hydroxylase protein family. The encoded phenylalanine hydroxylase enzyme hydroxylates phenylalanine to tyrosine and is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Aug 2017]
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; EC 1.14.16.1; Oxidoreductase. Chromosomal Location of Human Ortholog: 12q23.2. Cellular Component: cytosol; extracellular exosome. Molecular Function: iron ion binding; phenylalanine 4-monooxygenase activity. Biological Process: amino acid biosynthetic process; catecholamine biosynthetic process; L-phenylalanine catabolic process; neurotransmitter biosynthetic process. Disease: Phenylketonuria