Valerie Berthelier Assistant Professor, Department of Medicine, Graduate School of Medicine, Conformational Diseases and Therapeutics Research Laboratory http://gsm.utmck.edu/cdt/main.htm 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) UT Medical Center 1924 Alcoa Hwy R302 Knoxville, TN 37920 Phone: 865-544-8987 Fax: 865-544-9460 vberthel@mc.utmck.edu

Keywords:
Protein aggregation, amyloid and amyloid-like fibrils, polyglutamine, polyalanine, serpins, high-throughput screening, inhibitors, chemical compounds

Research Area:
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.

Selected Publications (of 13):

• Berthelier V. and Wetzel R. Screening for Modulators of Aggregation with a Microplate Elongation Assay. Methods in Enzymology. In press
  


• Williams, A.D., Sega, M., Chen, M., Ketherpal, I., Geva, M., Berthelier, V., Kaleta, D., Cook, K.D. and Wetzel, R. (2005) Structural properties of Abeta protofibrils stabilized by a small molecule. PNAS, USA, 102:7115-20
  


• Berthelier V. and Wetzel R. (2003) An assay for characterizing the in vitro kinetics of polyglutamine aggregation. Methods in Molecular Medicine: Neurogenetics. 217:293-303
  


• Chen S., Berthelier V., Hamilton J. B., O’Nuaillain B. and Wetzel R., (2002): Amyloid like features of polyglutamine aggregates and their assembly kinetics. Biochem. 41: 7391-7399.
  


• Berthelier V., Hamilton J. B., Chen S. and Wetzel R., (2001): A Microtiter Plate Assay for Polyglutamine Aggregate Extension. Anal. Biochem. 295:227-36.
  


• Chen S., Berthelier V., Yang W. and Wetzel R., (2001): Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity. J. Mol. Biol. 311:173-182.