Phenylalanine hydroxylase (PAH)
Cofactors used
Clinical / pharmacological. Phenylketonuria: musty odour, intellectual disability, hypopigmentation; BH4 defects cause malignant PKU
What it does, reaction by reaction
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion
L-phenylalanine + O₂ + tetrahydrobiopterin (BH₄) → L-tyrosine + H₂O + dihydrobiopterin (BH₂)
Converts Phenylalanine O2 Tetrahydrobiopterin (BH4) into Tyrosine H2O Dihydrobiopterin (BH2)
› Notes
Phenylalanine hydroxylase (PAH) catalyzes this irreversible hydroxylation; BH₄ is regenerated from BH₂ by dihydropteridine reductase using NADPH. This is the committed entry step for phenylalanine degradation and is also required to generate tyrosine when dietary tyrosine is insufficient.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples)
Listed there as: Phenylalanine hydroxylase
Phenylalanine (allosteric/substrate activation); BH₄ availability
BH₄ deficiency; inhibitory phosphorylation states/low substrate
Mainly substrate and cofactor regulated; chronic nutritional/hormonal effects are indirect
Recent literature
Europe PMC · fetched just now · sorted by publication date
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1
Advances and perspectives in genetically encoded biosensors for biomarker detection and theranostic circuits.
Xu J, Qi Q, Wang Q. · 2026-09-23
open access unreviewed -
2
Synchronous dual-Cas12a activation via dimeric palindromic hairpin-programmed cascade amplification for ultrasensitive molecular diagnostics.
Zhao J, Wang Y, Chen B, Guo L, Li H, Xue L, Xu J, Wu R. · 2026-08-24
unreviewed -
3
Estrogenic profiles in lungs from a rat model of pulmonary arterial hypertension measured by liquid chromatography triple quadrupole mass spectrometry.
Weatherill JW, Labazi H, Denver N, Simpson JP, Denham SG, Homer NZ, MacLean MR, Andrew R. · 2026-08-13
unreviewed -
4
Mechanisms of competitive adsorption of naphthalene, phenanthrene, and benzo[ghi]perylene on UV-aged polyvinyl chloride microplastics.
Li X, Long J, Qin Y, He J, Yang R, Dong G. · 2026-08-19
unreviewed -
5
Waterpipe smoking and risk of cardiovascular disease mortality: Findings from a Prospective Cohort Study.
Le NT, Nguyen HL, Le LT, Nguyen TG, Truong DT, Le PH, Wu L, Pham YT, Luu HN. · 2026-02-28
open access unreviewed -
6
Preliminary study of individualized therapy for ventricular septal defect with severe pulmonary arterial hypertension.
Tang W, Cai J, Zhang S, Li Y, Tang M, Zheng Z, Tu Z, Li X, Xu C, Chen W, Fan C, Yang J. · 2026-09-30
unreviewed -
7
Integrated risk prioritization of rubber additives based on hazard ranking and exposure characterization.
Wang S, Dai W, Wang B, Heng L. · 2026-09-02
open access unreviewed -
8
Lower blood phenylalanine concentrations may be of clinical benefit in adults with phenylketonuria.
Lubout CMA, van Wegberg AMJ, van Adrichem-van Dam E, Jansen ME, van Steenis EM, Heiner-Fokkema MR, van Sprons… · 2026-09-17
open access unreviewed -
9
Sleep-disordered breathing in patients with group 1 and group 4 pulmonary hypertension.
Yüce K, Aşkar S, Aşkar M. · 2026-09-09
open access unreviewed -
10
Phloretin as a functional feed bioactive in livestock: Biological role, biosynthesis, and practical applications.
Areb E, Zhu Q, Han L, Pan C, Li W, Lan X. · 2026-07-25
open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.