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Protein Page:
PAH (human)

Overview
PAH phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; EC 1.14.16.1; Oxidoreductase
Chromosomal Location of Human Ortholog: 12q22-q24.2
Cellular Component: cytosol
Molecular Function: phenylalanine 4-monooxygenase activity
Biological Process: amino acid biosynthetic process; L-phenylalanine catabolic process
Disease: Phenylketonuria
Reference #:  P00439 (UniProtKB)
Alt. Names/Synonyms: PAH; PH; PH4H; Phe-4-monooxygenase; phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1
Gene Symbols: PAH
Molecular weight: 51,862 Da
Basal Isoelectric point: 6.15  Predict pI for various phosphorylation states
Select Structure to View Below

PAH

Protein Structure Not Found.
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