Human Phenylalanine-4-hydroxylase, PAH ELISA Kit

SKU: E6875Hu

Size:96 Tests-1 Kit

Background: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.

Research_area:Signal Transduction; Metabolism

Applications:ELISA

Target_protein:PAH

Reactivity:Human

Assay type:Sandwich

Sensitivity:4.86ng/L

Detection range:10-2000ng/L

Sample type:Serum, plasma or other biological fluids

Full product name:Phenylalanine-4-hydroxylase

Species:Human

Storage:2-8ºC

Assay time:1h 30m

UniProt accession:P00439

MASS(Da):51,862

GeneID:5053

Synonyms:PAH;PH;Phe-4-monooxygenase;Phenylalanine-4-hydroxylase;PKU;PKU1

Gene names:PAH

Standard solution:2400ng/L