PAH

Phenylalanine hydroxylase, metabolizes phenylalanine.

Key Functions:

• Converts phenylalanine to tyrosine.
• Linked to phenylketonuria (PKU).

Interacting Systems & Components:

• Metabolic System (amino acid metabolism)
• BH4 (cofactor)
• Hepatocyte (expression site)

Gene Record

Approved symbol: PAH

Approved name: phenylalanine hydroxylase

Locus: 12q23.2

Locus type: gene with protein product

HGNC: HGNC:8582

From the HGNC naming authority.