Part:BBa_K200008

Phenylalanine hydroxylase

[http://en.wikipedia.org/wiki/Phenylalanine_hydroxylase Phenylalanine hydroxylase (PAH)] is the enzyme that breaks down [http://en.wikipedia.org/wiki/Phenylalanine phenylalanine] to [http://en.wikipedia.org/wiki/Tyrosine tyrosine]. Deficiency of this enzyme activity results in the autosomal recessive disorder [http://en.wikipedia.org/wiki/Phenylketonuria phenylketonuria].#PAH1

Usage and Biology

PAH works well at a PH of around 7.0 (similar to the small intestine), and it is a non-heme iron-dependent enzyme. The enzyme binds three substrates: reduced pterin (PH4), phenylalanine, and oxygen, and releases two products: tyrosine and oxidized pterin #PAH2. The enzyme is stable between 7 and 40 degrees celsius#PAH2 and in this range activity increases exponentially with temperature but Km (Michaelis-Menten constant) does not vary significantly between 20 and 40 degrees. The enzyme can be rendered protease resistant via full phosphorylation.#PAH3

Furthermore, the presence of oxygen and an additional cofactor (TPNH) is required for the hydrolysis to take place #PAH4.

Requirements

• Works for PH 7
• Works between 7 to 40 degrees

Sequence and Features

Assembly Compatibility:

• 10
  COMPATIBLE WITH RFC[10]
• 12
  COMPATIBLE WITH RFC[12]
• 21
  INCOMPATIBLE WITH RFC[21]

  Illegal BglII site found at 287
  Illegal BamHI site found at 814
  Illegal XhoI site found at 524
  
• 23
  COMPATIBLE WITH RFC[23]
• 25
  COMPATIBLE WITH RFC[25]
• 1000
  COMPATIBLE WITH RFC[1000]

References

[http://www.ncbi.nlm.nih.gov/nuccore/NM_000277.1?report=fasta Sequence from NCBI]

<biblio>

1. PAH1 pmid=2014036
2. PAH2 pmid=15708798
3. PAH3 pmid=8573072
4. PAH4 pmid=13525410

</biblio>