Recombinant Human PAH/PH Protein (415 Asn/Asp, His Tag)

Specifications

Catalog number

PKSH030912

Size / count

50 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

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.

Synonyms

PH; PKU; PKU1

UniProt

P00439

Accession Number

P00439

Expression System

Baculovirus-Insect Cells

Tag

N-His

Sequence

Met 1-Lys 452

Field of Research

Signal Transduction; metabolism

Endotoxin

< 1.0 EU per μg of the protein as determined by the LAL method.

Purity

> 70 % as determined by reducing SDS-PAGE.

Bioactivity

Not validated for activity

Reconstitution

Please refer to the printed manual for detailed information.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Calculated Molecular Weight

54.0 kDa

Observed Molecular Weight

50 kDa

Species

Human