Communication

Structural and Mechanistic Basis of the Interaction between a Pharmacological Chaperone and Human Phenylalanine Hydroxylase

  1. Renzo Torreblanca1,
  2. Erandi Lira-Navarrete2,
  3. Prof. Javier Sancho1,*,
  4. Dr. Ramon Hurtado-Guerrero2,*

Article first published online: 30 APR 2012

DOI: 10.1002/cbic.201200188

Issue

ChemBioChem

ChemBioChem

Volume 13, Issue 9, pages 1266–1269, June 18, 2012

Additional Information

How to Cite

Torreblanca, R., Lira-Navarrete, E., Sancho, J. and Hurtado-Guerrero, R. (2012), Structural and Mechanistic Basis of the Interaction between a Pharmacological Chaperone and Human Phenylalanine Hydroxylase. ChemBioChem, 13: 1266–1269. doi: 10.1002/cbic.201200188

Author Information

  1. 1

    Department of Biochemistry and Molecular and Cellular Biology, Institute of Biocomputation and Physics of Complex Systems (BIFI), University of Zaragoza, BIFI-IQFR (CSIC) Joint Unit, Pedro Cerbuna 12, Mariano Esquillor s/n, Campus Rio Ebro, Edificio I+D

  2. 2

    Fundacion ARAID, Edificio Pignatelli 36, Institute of Biocomputation and Physics of Complex Systems (BIFI), University of Zaragoza, BIFI-IQFR (CSIC) Joint Unit, Mariano Esquillor s/n, Campus Rio Ebro, Edificio I+D

*Department of Biochemistry and Molecular and Cellular Biology, Institute of Biocomputation and Physics of Complex Systems (BIFI), University of Zaragoza, BIFI-IQFR (CSIC) Joint Unit, Pedro Cerbuna 12, Mariano Esquillor s/n, Campus Rio Ebro, Edificio I+D

Publication History

  1. Issue published online: 13 JUN 2012
  2. Article first published online: 30 APR 2012
  3. Manuscript Received: 19 MAR 2012

Funded by

  • Fundación Agencia Aragonesa para la Investigación y el Desarrollo (ARAID, Spain)
  • Ministerio de Ciencia e Innovación (MICINN, Spain). Grant Numbers: BFU2010-19504, BFU2010-16297
  • Grupo Protein Targets B89

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Keywords:

  • folding mechanisms;
  • pharmacological chaperones;
  • phenylalanine hydroxylases;
  • phenylketonuria
Thumbnail image of graphical abstract

Not without a chaperone: Pharmacological chaperones are designed to bind and ideally stabilise their target protein. Here, we elucidate the molecular mechanism of a potential pharmacological chaperone to treat phenylketonuria. The crystal structure of human phenylalanine hydroxylase with compound IV may help in the rational design of more efficient compounds to treat this disease.

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