catalog number :
MBS2010263
products type :
Recombinant Protein
products full name :
Recombinant Phenylalanine Hydroxylase (PAH)
products short name :
[Phenylalanine Hydroxylase (PAH)]
other names :
[phenylalanine-4-hydroxylase; Phenylalanine-4-hydroxylase; phenylalanine-4-hydroxylase; phe-4-monooxygenase; phenylalanine 4-monooxygenase; phenylalanine hydroxylase; Phe-4-monooxygenase]
products gene name :
[PAH]
other gene names :
[PAH; PAH; PH; PKU; PKU1; PAH]
uniprot entry name :
PH4H_HUMAN
concentration :
Original Concentration: 200 ug/mL
storage stability :
Storage: Avoid repeated freeze/thaw cycles. Store at 2-8ºC for one month. Aliquot and store at -80ºC 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 :
Positive Control; Immunogen; SDS-PAGE; WB. (May be suitable for use in other assays to be determined by the end user.)
image1 heading :
SDS-Page
other info1 :
Organism Species: Homo sapiens (Human). Source: Prokaryotic expression. Residues: Met1~Gln226. Tags: N-terminal His Tag. Subcellular Location: Extracellular matrix. Traits: Freeze-dried powder. Buffer: 100mM NaHCO3, 500mM NaCl, pH8.3, containing 0.01% sarcosyl,
5%Trehalose.
other info2 :
Predicted isoelectric point: 6.6. Predicted Molecular Mass: 27.6kDa. Accurate Molecular Mass: 28kDa as determined by SDS-PAGE reducing conditions. Usage: Reconstitute in 100mM NaHCO3, 500mM NaCl (pH8.3) to a concentration of 0.1-1.0 mg/mL. Do
not vortex.
ncbi acc num :
NP_000268.1
ncbi gb acc num :
NM_000277.1
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); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Phenylalanine And Tyrosine Catabolism Pathway (106189); Phenylalanine Metabolism Pathway (82960); Phenylalanine Metabolism Pathway (327)
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: 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; 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