Published September 21, 2026
| Version v1
Poster
Open
BRD4780-mediated clearance of mutant alpha-1-antitrypsin involves the autophagiclysosomal pathway
Authors/Creators
-
1.
Institute of Clinical and Experimental Medicine
- 2. Research Unit for Rare Diseases, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic
- 3. Medical Clinic III, Gastroenterology, Metabolic Diseases and Intensive Care, University Hospital RWTH Aachen, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE LIVER), Aachen, Germany
Description
Accumulation of misfolded proteins is a hallmark of several proteinopathies and represents a potential therapeutic target. In alpha-1-antitrypsin deficiency (AATD), mutant A1AT accumulates intracellularly due to protein misfolding. BRD4780 has been shown to facilitate the degradation of proteins retained within the early secretory pathway. We investigated its effect on mutant A1AT and the potential involvement of autophagy in this process.
Notes
Supported by the Ministry of Health of the Czech Republic in cooperation with the Czech Health
Research Council within the National Institute CarDia, project No. NW26A-CARDIA.
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FEBS_2026_Veselá_Abstract.pdf
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