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- W2004644004 endingPage "1052" @default.
- W2004644004 startingPage "1030" @default.
- W2004644004 abstract "Conformational diseases are often caused by mutations, altering protein folding and stability in vivo. We review here our recent work on the effects of mutations on the human phosphoglycerate kinase 1 (hPGK1), with a particular focus on thermodynamics and kinetics of protein folding and misfolding. Expression analyses and in vitro biophysical studies indicate that disease-causing mutations enhance protein aggregation propensity. We found a strong correlation among protein aggregation propensity, thermodynamic stability, cooperativity and dynamics. Comparison of folding and unfolding properties with previous reports in PGKs from other species suggests that hPGK1 is very sensitive to mutations leading to enhance protein aggregation through changes in protein folding cooperativity and the structure of the relevant denaturation transition state for aggregation. Overall, we provide a mechanistic framework for protein misfolding of hPGK1, which is insightful to develop new therapeutic strategies aimed to target native state stability and foldability in hPGK1 deficient patients." @default.
- W2004644004 created "2016-06-24" @default.
- W2004644004 creator A5052018914 @default.
- W2004644004 creator A5065820626 @default.
- W2004644004 creator A5085019663 @default.
- W2004644004 date "2013-12-18" @default.
- W2004644004 modified "2023-09-30" @default.
- W2004644004 title "Protein Stability, Folding and Misfolding in Human PGK1 Deficiency" @default.
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