PAH

PAH is 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.
Full Name
Phenylalanine Hydroxylase
Function
Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.
Biological Process
Catecholamine biosynthetic process1 PublicationNAS:BHF-UCL
Cellular amino acid biosynthetic processManual Assertion Based On ExperimentTAS:ProtInc
L-phenylalanine catabolic processIEA:UniProtKB-UniPathway
Neurotransmitter biosynthetic process1 PublicationNAS:BHF-UCL
Tyrosine biosynthetic processManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Cytosol
Involvement in disease
Phenylketonuria (PKU):
Autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA):
Mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
Hyperphenylalaninemia (HPA):
Mildest form of phenylalanine hydroxylase deficiency.
PTM
Phosphorylation at Ser-16 increases basal activity and facilitates activation by the substrate phenylalanine.
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Anti-PAH antibodies

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Mouse Anti-PAH Recombinant Antibody (CBYC-P139) (CBMAB-P0678-YC)

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Target: PAH
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBYC-P139
Application*: WB, IC, P, C, E
Datasheet SDS

Rabbit Anti-PAH Recombinant Antibody (CBYC-P138) (CBMAB-P0677-YC)

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Target: PAH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBYC-P138
Application*: IC, IF
Datasheet SDS

Mouse Anti-PAH Recombinant Antibody (717CT21.1.1) (CBMAB-P0676-YC)

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Target: PAH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human, Mouse, Rat
Clone: 717CT21.1.1
Application*: WB, E, IH
Datasheet SDS

Human PAH ELISA Kit (V2LY-0626-LY5711)

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Target: PAH
Sensitivity: 0.0049 ng/mL
Detection Range: 0.01-2 ng/mL
Sample Type: Serum, Plasma, cell culture supernates
Specificity: Human
Assay Type: Sandwich
Reactivity: Human
Datasheet SDS

Mouse Pah ELISA Kit (V2LY-0626-LY2865)

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Target: Pah
Sensitivity: 0.0079 ng/mL
Detection Range: 0.015-3 ng/mL
Sample Type: Serum, Plasma, cell culture supernates
Specificity: Mouse
Assay Type: Sandwich
Reactivity: Mouse
Datasheet SDS

Human Recombinant PAH protein, His Tag (V2LY-0526-LY5947)

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Target: PAH
Expressed Host: Baculovirus-Insect Cells
Sequence: Amino Acid: 1-452, 415/
Tag: His Tag
Datasheet SDS

Cynomolgus Recombinant PAH protein, His Tag (V2LY-0526-LY1618)

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Target: PAH
Expressed Host: Baculovirus-Insect Cells
Sequence: Amino Acid: 1-437
Tag: His Tag
Datasheet SDS

Rabbit Anti-PAH Recombinant Antibody (
17D10) (V2LY-0725-LY991)

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Target: PAH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 17D10
Application*: E, IH, IF
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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