Stimulation of hepatic phenylalanine hydroxylase activity but not Pah-mRNA expression upon oral loading of tetrahydrobiopterin in normal mice.

@article{Scavelli2005StimulationOH,
  title={Stimulation of hepatic phenylalanine hydroxylase activity but not Pah-mRNA expression upon oral loading of tetrahydrobiopterin in normal mice.},
  author={Rossana Scavelli and Zhaobing Ding and Nenad Blau and Jan Haavik and Aurora Martinez and Beat Th{\"o}ny},
  journal={Molecular genetics and metabolism},
  year={2005},
  volume={86 Suppl 1},
  pages={
          S153-5
        }
}
Tetrahydrobiopterin (BH4) supplementation in patients with BH4-responsive phenylalanine hydroxylase (PAH) deficiency is an alternative to low-phenylalanine diet. To further investigate hepatic BH4-responsiveness, oral administration of 50 mg BH4/kg/day for 5 weeks was performed in wild-type mice. We observed a 2-fold increase in PAH protein by quantitative Western blot analysis and a 1.7-fold increase in enzyme activity, but no change in Pah-mRNA expression by quantitative real-time PCR… CONTINUE READING
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The fact of intravenously administered tetrahydrobiopterin and its implications for heterologous gene therapy of phenylketonuria

  • C. O. Harding, M. NeV, +4 authors S. Milstien
  • Mol. Genet. Metab. 81
  • 2004
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