BRD4780-mediated clearance of mutant alpha-1-antitrypsin involves the autophagiclysosomal pathway

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.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2026-09-21
DOI
https://doi.org/10.5281/zenodo.22870510
Primary Topic
Endoplasmic Reticulum Stress and Disease
Type
article
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article

BRD4780-mediated clearance of mutant alpha-1-antitrypsin involves the autophagiclysosomal pathway

V. Barešová, Šárka Veselá, Mariia Lunová, Milan Jirsa et al.
Zenodo (CERN European Organization for Nuclear Research)
Endoplasmic Reticulum Stress and Disease
article

BRD4780-mediated clearance of mutant alpha-1-antitrypsin involves the autophagiclysosomal pathway

V. Barešová, Šárka Veselá, Mariia Lunová, Milan Jirsa, M. Zivna, Strnad P., S. Kmoch, P. Vyleťal, D. Mušálková
article en

Abstract

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.

Zenodo (CERN European Organization for Nuclear Research)
Charles University (CZ), Institute of Clinical and Experimental Medicine (CZ), General University Hospital in Prague (CZ), RWTH Aachen University (DE)
Good health and well-being
Openalex Percentile: Top 14%
Endoplasmic Reticulum Stress and Disease
DOIOpen Access PDFOpenAlex
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BRD4780-mediated clearance of mutant alpha-1-antitrypsin involves the autophagiclysosomal pathway — V. Barešová, Šárka Veselá, et al. · Zenodo (CERN European Organization for Nuclear Research) (2026) | TGRS Research Map | TGRS