| 1 |
35356682 | Development of an mRNA replacement therapy for phenylketonuria. | 2022 Jun 14 |
3 |
| 2 |
35600090 | Lipid nanoparticle delivers phenylalanine ammonia lyase mRNA to the liver leading to catabolism and clearance of phenylalanine in a phenylketonuria mouse model. | 2022 Sep |
4 |
| 3 |
33824313 | The Pah-R261Q mouse reveals oxidative stress associated with amyloid-like hepatic aggregation of mutant phenylalanine hydroxylase. | 2021 Apr 6 |
5 |
| 4 |
33896013 | Use of an adeno-associated virus serotype Anc80 to provide durable cure of phenylketonuria in a mouse model. | 2021 Nov |
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| 5 |
34819582 | Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria. | 2021 Nov 24 |
3 |
| 6 |
29514280 | Secondary BH4 deficiency links protein homeostasis to regulation of phenylalanine metabolism. | 2018 May 15 |
6 |
| 7 |
28520731 | Genetically engineered probiotic for the treatment of phenylketonuria (PKU); assessment of a novel treatment in vitro and in the PAHenu2 mouse model of PKU. | 2017 |
1 |
| 8 |
26822703 | DNA methylation in the pathophysiology of hyperphenylalaninemia in the PAH(enu2) mouse model of phenylketonuria. | 2016 Sep |
1 |
| 9 |
27091224 | Phenylketonuria: Direct and indirect effects of phenylalanine. | 2016 Jul |
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| 10 |
25296915 | Hippocampal synaptic connectivity in phenylketonuria. | 2015 Feb 15 |
2 |
| 11 |
25664353 | Identification of a biochemical marker for endothelial dysfunction using Raman spectroscopy. | 2015 Apr 7 |
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| 12 |
23867524 | Erythrocytes encapsulated with phenylalanine hydroxylase exhibit improved pharmacokinetics and lowered plasma phenylalanine levels in normal mice. | 2013 Aug |
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| 13 |
22644647 | The mechanism of BH4 -responsive hyperphenylalaninemia--as it occurs in the ENU1/2 genetic mouse model. | 2012 Oct |
7 |
| 14 |
20887755 | Phenylalanine hydroxylase (PAH) from the lower eukaryote Leishmania major. | 2011 Jan |
2 |
| 15 |
21917493 | Hepatocytes from wild-type or heterozygous donors are equally effective in achieving successful therapeutic liver repopulation in murine phenylketonuria (PKU). | 2011 Nov |
2 |
| 16 |
19916803 | Comparison of adeno-associated virus pseudotype 1, 2, and 8 vectors administered by intramuscular injection in the treatment of murine phenylketonuria. | 2010 Apr |
1 |
| 17 |
20151201 | Long-term correction of murine phenylketonuria by viral gene transfer: liver versus muscle. | 2010 Dec |
2 |
| 18 |
20943816 | Metabolomics identifies novel Hnf1alpha-dependent physiological pathways in vivo. | 2010 Dec |
1 |
| 19 |
19172175 | Carcinogenic effects in a phenylketonuria mouse model. | 2009 |
1 |
| 20 |
19560382 | In vivo regulation of phenylalanine hydroxylase in the genetic mutant hph-1 mouse model. | 2009 Nov |
3 |
| 21 |
18203898 | Dietary glycomacropeptide supports growth and reduces the concentrations of phenylalanine in plasma and brain in a murine model of phenylketonuria. | 2008 Feb |
1 |
| 22 |
17112485 | PKU is a reversible neurodegenerative process within the nigrostriatum that begins as early as 4 weeks of age in Pah(enu2) mice. | 2007 Jan 5 |
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| 23 |
17565982 | A novel approach for enzyme replacement therapy. The use of phenylalanine hydroxylase-based fusion proteins for the treatment of phenylketonuria. | 2007 Aug 10 |
1 |
| 24 |
15863237 | Expression of calpastatin, minopontin, NIPSNAP1, rabaptin-5 and neuronatin in the phenylketonuria (PKU) mouse brain: possible role on cognitive defect seen in PKU. | 2005 Jun |
2 |
| 25 |
15925112 | Altered expression of myocilin in the brain of a mouse model for phenylketonuria (PKU). | 2005 Jul 15 |
5 |
| 26 |
15946242 | Characterization of transgenic mice with the expression of phenylalanine hydroxylase and GTP cyclohydrolase I in the skin. | 2005 Jul |
1 |
| 27 |
16043102 | Low therapeutic threshold for hepatocyte replacement in murine phenylketonuria. | 2005 Aug |
6 |
| 28 |
15464429 | Wild-type phenylalanine hydroxylase activity is enhanced by tetrahydrobiopterin supplementation in vivo: an implication for therapeutic basis of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency. | 2004 Sep-Oct |
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| 29 |
8828602 | Gene therapy for phenylketonuria. | 1996 Jul |
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| 30 |
8965095 | Evidence for central nervous system glial cell plasticity in phenylketonuria. | 1996 Jul |
2 |
| 31 |
7584088 | Gene therapy for phenylketonuria: phenotypic correction in a genetically deficient mouse model by adenovirus-mediated hepatic gene transfer. | 1994 Jul |
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| 32 |
7766948 | Gene therapy for phenylketonuria. | 1994 Dec |
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| 33 |
1312261 | Reconstitution of enzymatic activity in hepatocytes of phenylalanine hydroxylase-deficient mice. | 1992 Jan |
2 |
| 34 |
1321825 | Tissue- and development-specific expression of the human phenylalanine hydroxylase/chloramphenicol acetyltransferase fusion gene in transgenic mice. | 1992 Jul 25 |
2 |
| 35 |
3741696 | Induction of hyperphenylalaninemia in mice by ethionine and phenylalanine. | 1986 Aug |
1 |
| 36 |
6476382 | Determination of phenylalanine hydroxylase activity by liquid chromatography/electrochemistry. | 1984 Jun |
1 |
| 37 |
7150251 | The effects of chronic hyperphenylalaninaemia on mouse brain protein synthesis can be prevented by other amino acids. | 1982 Aug 15 |
1 |
| 38 |
153135 | Effects of p-chlorophenylalanine and alpha-methylphenylalanine on amino acid uptake and protein synthesis in mouse neuroblastoma cells. | 1978 Sep 15 |
1 |
| 39 |
566796 | Free amino acid pool in the brain of mice homozygous for the gene "dilute lethal". | 1977 |
1 |