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Valerie Berthelier

Assistant Professor

UT Medical Center


Research

Our laboratory specializes in finding small molecule therapeutics that prevent or significantly slow down diseases caused by unwanted polymerization, aggregation or fibril formation of proteins. Such diseases, also referred to as Conformational disorders, remain virtually untreatable. Through our research on Oculopharyngeal Muscular dystrophy, Antitrypsin deficiency, Alzheimer?s and Huntington?s diseases, we are not only searching for small therapeutic agents but also trying to identify the various mechanisms and cytotoxic pathways responsible for the disease.

Description of Research

Small molecules as modulators of polyglutamine aggregation

The major goal of this project is to identify small chemical molecules that can affect the conformation of polyglutamine aggregates and lead to the discovery of potential treatment. Structure and Biophysics of Intrinsically Disordered Proteins. Scientific research and development involving the production, purification, and biophysical characterization of intrinsically disordered proteins for neutron scattering studies at ORNL


Education

  • PhD: Molecular Biophysics, University of Paris 7 Denis Diderot, France (1998)
  • Diploma of Graduate Study in Molecular Biophysics, University of Paris, France (1993)
  • MS: Biochemistry, University of Paris 7 Denis Diderot, France (1992)
  • BS: Biology, University of Paris 13 Bobigny, France (1991)

Publications

Investigating the structural impact of the glutamine repeat in huntingtin assembly. Perevozchikova T, Stanley CB, McWilliams-Koeppen HP, Rowe EL, Berthelier V. Biophys J. 2014 Jul 15;107(2):411-21. doi: 10.1016/j.bpj.2014.06.002.

Structural formation of huntingtin exon 1 aggregates probed by small-angle neutron scattering. Stanley CB, Perevozchikova T, Berthelier V. Biophys J. 2011 May 18;100(10):2504-12.

Imaging polyglutamine deposits in brain tissue. Osmand AP, Berthelier V., Wetzel R. Methods Enzymol. 2006;412:106-22. Review.

Screening for modulators of aggregation with a microplate elongation assay. Berthelier V., Wetzel R. Methods Enzymol. 2006;413:313-25.

Structural properties of Abeta protofibrils stabilized by a small molecule. Williams AD, Sega M, Chen M, Kheterpal I, Geva M, Berthelier V., Kaleta DT, Cook KD, Wetzel R. Proc Natl Acad Sci U S A. 2005 May 17;102(20):7115-20. Epub 2005 May 9.

An assay for characterizing in vitro the kinetics of polyglutamine aggregation. Berthelier V., Wetzel R. Methods Mol Biol. 2003;217:295-303. No abstract available.

Amyloid-like features of polyglutamine aggregates and their assembly kinetics. Chen S, Berthelier V., Hamilton JB, O'Nuallain B, Wetzel R. Biochemistry. 2002 Jun 11;41(23):7391-9.


Contact Information

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