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
Interpol review of forensic fire debris analysis and fire investigation, 2023-2025.
Evans M. · 2026-06-15
open access unreviewed -
2
Associations between growth, maturation and non-contact injury risk in Scottish male academy football: a prospective multi-academy study.
Curnyn S, Leslie M, Cumming S, Palmer D, Williams S. · 2026-06-02
unreviewed -
3
Identification risk factors for perinatal complications among pregnant women with pulmonary arterial hypertension.
Wu Y, Zhu Y, Gao G, Li S, Chen Y, Zhang L. · 2026-05-04
unreviewed -
4
Prognostic value of peak tricuspid regurgitation velocity in hospitalized patients with heart failure with preserved ejection fraction: an multi-institutional study.
Chang CY, Chen CC, Tsai ML, Hsieh MJ, Lee CH, Chen TH, Chen SW, Chang SH, Chu PH, Hsieh IC, Wen MS, Chen DY. · 2026-04-10
open access unreviewed -
5
Loss of productivity among commercially insured patients with pulmonary arterial hypertension in the United States.
Watzker A, Ferro C, Dieguez G, Girdish C, Alsumali A, Lautsch D, El-Kersh K. · 2026-03-20
unreviewed -
6
Smoking exposure on diagnosis and survival of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Data from REHAP registry.
Khilzi K, Blanco I, Piccari L, Barberà JA, López-Reyes R, Otero-González I, Alcolea S, Ribas J, Álvarez-Barre… · 2026-03-10
unreviewed -
7
The process of developing an HIV disclosure intervention for youth with perinatally acquired HIV: The HIV Empowering Adults' Decisions to Share - UK/Uganda Project (Heads-Up).
Evangeli M, Gnan G, Musiime V, Fidler S, Seeley J, Frize G, Uwizera A, Robinson J, Foster C. · 2026-03-09
unreviewed -
8
Bulk and single-cell transcriptomics for identification of potential diagnostic biomarkers associated with pulmonary arterial hypertension and integrated stress response and experimental validation.
Li H, Li M, Qiu C, Yang Y, Lan C. · 2026-02-18
unreviewed -
9
The TRAP complex (SSR1-SSR4): mechanistic roles and therapeutic opportunities.
Zhang J, Wan X, Gong A. · 2026-02-06
open access unreviewed - 10
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