Publication Date:
2015
abstract:
Thermal shift assay can be used for the high-throughput screening of pharmacological chaperones. These drugs are small molecules
that bind a mutant protein and stabilize it. We demonstrated the robustness, reproducibility and versatility of the method
using two molecules that are in clinical trial for Fabry or Pompe disease, Deoxygalactonojirimycin and N-Butyldeoxynojirimycin,
and their target enzymes, lysosomal alpha-galactosidaseA and alpha-glucosidase, as test cases. We assessed the influence of solvents
and of scanning rate on the measures. We showed that a value that is equivalent to the melting temperature can be obtained
by the first derivatives of raw data. We discuss the advantages of the method and the precaution to be taken in running
the experiments.
Iris type:
01.01 Articolo in rivista
Keywords:
rare disease; pharmacological chaperones; thermal shift assay
List of contributors: