Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Rudashevskaya, EL; Stockner, T; Trauner, M; Freissmuth, M; Chiba, P.
Pharmacological correction of misfolding of ABC proteins.
Drug Discov Today Technol. 2014; 12(12):e87-e94
Doi: 10.1016/j.ddtec.2014.03.009
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- Co-Autor*innen der Med Uni Graz
- Trauner Michael
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- Abstract:
- The endoplasmic reticulum (ER) quality control system distinguishes between correctly and incorrectly folded proteins to prevent processing of aberrantly folded conformations along the secretory pathway. Non-synonymous mutations can lead to misfolding of ABC proteins and associated disease phenotypes. Specific phenotypes may at least partially be corrected by small molecules, so-called pharmacological chaperones. Screening for folding correctors is expected to open an avenue for treatment of diseases such as cystic fibrosis and intrahepatic cholestasis.
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