1. We propose that CNS monoamine deficiency may be the cause of the partially reversible adverse behavioral effects associated with chronic HPA in Pah(enu2) mice, but that phenylalanine-reducing treatments initiated during adulthood are unable to correct the neuropathology and attendant cognitive deficits that develop during juvenile life in late-treated Pah(enu2/enu2) mice PMID: 29331172
2. we assessed DNA methylation patterns in brain tissues using methylated DNA immunoprecipitation and paired end sequencing in adult PAH(enu2) animals maintained under either continuous dietary Phe restriction or chronic hyperphenylalaninemia. Heterozygous PAH(enu2/WT) litter mates served as controls for normal Phe exposure PMID: 26822703
3. These observations support the proposition that differences in phenylalanine hydroxylase activity underlie the variation in S-carboxymethyl-L-cysteine sulfoxidation and that no other enzyme is able to undertake this reaction. PMID: 26338263
4. A single injection of PAH-RBCs was able to decrease Phe levels by nearly 80% in normal mice PMID: 23867524
5. high levels of Phe, such as in phenylketonuria, are unlikely to directly induce brain hypomyelination, suggesting involvement of alternative mechanisms in this myelination defect PMID: 20151197
6. phenylalanine concentration regulates the activity of PAH in the hph-1 mouse and that this acts via a mechanism that includes phosphorylation of the PAH molecule PMID: 19560382
7. Tetrahydrobiopterin protects PAH activity in vivo. PMID: 15556637
8. low level of phenylalanine clearance was observed in mice expressing PAH and GTP-CH from the keratin 14 promoter PMID: 15946242

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KEGG: mmu:18478

STRING: 10090.ENSMUSP00000020241

UniGene: Mm.263539