E6875Hu
Human Phenylalanine-4-hydroxylase, PAH ELISA Kit
Bioassay Technology Laboratory
DESCRIPTION
Phenylalanine hydroxylase (PAH) (EC 1.14.16.1) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PAH is one of three members of the biopterin-dependent aromatic amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During the reaction, molecular oxygen is heterolytically cleaved with sequential incorporation of one oxygen atom into BH4 and phenylalanine substrate. In humans, mutations in its encoding gene, PAH, can lead to the metabolic disorder phenylketonuria.
DETAILS
- GeneID: 5053
- Species: Human
- Storage: 2-8ºC
- MASS(Da): 51,862
- Synonyms: PAH;PH;Phe-4-monooxygenase;Phenylalanine-4-hydroxylase;PKU;PKU1
- Assay Time: 1h 30m
- Assay Type: Sandwich
- Gene Names: PAH
- Reactivity: Human
- Sample Type: Serum, plasma, cell culture supernates
- Sensitivity: 4.86ng/L
- Target Protein: PAH
- Detection Range: 10-2000ng/L
- Full Product Name: Phenylalanine-4-hydroxylase
- Standard Solution: 2400ng/L
- UniProt Accession: P00439