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Article

In vivo regulation of phenylalanine hydroxylase in the genetic mutant hph-1 mouse model.

Molecular genetics and metabolism - 1 Nov 2009

Gunasekera Richard S, Hyland Keith

Abstract excerpt

The hph-1 mouse has low liver activity of GTP cyclohydrolase 1, the rate limiting enzyme in the biosynthesis of tetrahydrobiopterin (BH(4)). BH(4) is the cofactor for phenylalanine hydroxylase (PAH) and in the early stages of life the hph-1 mouse is hyperphenylalaninemic. At approximately 15 days after birth the blood phenylalanine levels normalize. During this period the animals provide an in vivo model which...

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Source

Identifiers

PMID: 19560382
DOI: 10.1016/j.ymgme.2009.05.007

Genes

PAH

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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