Part:BBa_K200008
Phenylalanine hydroxylase
[http://en.wikipedia.org/wiki/Phenylalanine_hydroxylase Phenylalanine hydroxylase (PAH)] is the enzyme that determines the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder [http://en.wikipedia.org/wiki/Phenylketonuria phenylketonuria].#PAH1
The action of PAH is as follows:
[http://en.wikipedia.org/wiki/Phenylalanine Phenylalanine] is broken down to [http://en.wikipedia.org/wiki/Tyrosine tyrosine], which is an essential step in the production of [http://en.wikipedia.org/wiki/Catecholamine catecholamines].
PAH works well at a PH of around 7.4, and is denatured at temperatures above 30 degrees celsius (in experiments with gram-negative bacteria)#PAH2
Sequence and Features
- 10COMPATIBLE WITH RFC[10]
- 12COMPATIBLE WITH RFC[12]
- 21INCOMPATIBLE WITH RFC[21]Illegal BglII site found at 287
Illegal BamHI site found at 814
Illegal XhoI site found at 524 - 23COMPATIBLE WITH RFC[23]
- 25COMPATIBLE WITH RFC[25]
- 1000COMPATIBLE WITH RFC[1000]
References
[http://www.ncbi.nlm.nih.gov/nuccore/NM_000277.1?report=fasta Sequence from NCBI] <biblio>#PAH1 pmid=2014036 </biblio> <biblio>#PAH2 pmid=15708798 </biblio> <biblio>#PAH3 pmid=13525410 </biblio>
None |