背景：PAH （phenylalanine hydroxylase）, also known as PH, belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
It contains 1 ACT domain, N-terminal region of PAH is thought to contain allosteric binding sites for phenylalanine and to constitute an "inhibitory" domain that regulates the activity of a catalytic domain in the C-terminal portion of the molecule.
In humans, PAH is expressed both in the liver and the kidney, and there is some indication that it may be differentially regulated in these tissues.
PAH catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine.
It is one of three members of the pterin-dependent amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin and a non-heme iron for catalysis.
Defects in PAH are the cause of phenylketonuria （PKU）.
PKU is an 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.
仕様
サイズ：50ug
種由来：Human
発現宿主：Baculovirus-Insect Cells
純度：＞70 ％ as determined by reducing SDS-PAGE.
エンドトキシン濃度：＜1.0 EU per μg of the protein as determined by the LAL method.
アクセッションNo.：P00439
アミノ酸配列：Met 1-Lys 452
遺伝子構造：A DNA sequence encoding the human PAH （P00439） （Met 1-Lys 452） （415 Asn/Asp） was expressed, with a polyhistidine tag at the N-terminus.
保存条件：Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at ＜ -20℃ for 3 months.
