Article
Effect of pharmacological chaperones on brain tyrosine hydroxylase and tryptophan hydroxylase 2.
Journal of neurochemistry - 1 Aug 2010
Calvo Ana C, Scherer Tanja, Pey Angel L, Ying Ming, Winge Ingeborg, McKinney Jeffrey, Haavik Jan, Thöny Beat, Martinez Aurora
Abstract excerpt
Phenylalanine hydroxylase (PAH), tyrosine hydroxylase (TH) and the tryptophan hydroxylases (TPH1 and TPH2) are structurally and functionally related enzymes that share a number of ligands, such as amino acid substrates, pterin cofactors and inhibitors. We have recently identified four compounds (I-IV) with pharmacological chaperone effect for PAH and phenylketonuria mutants (Pey et al. (2008) J. Clin. Invest....
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