Evidence map›Paper›PMID 42567518›Full record

ArticleThe Journal of biological chemistry2026

p62/SQSTM1-KEAP1 complex prevents clearance of ubiquitinated Z alpha-1 antitrypsin and aggravates liver proteotoxicity.

Nunzia Pastore, Sergio Attanasio, Francesco Annunziata, Claudia D'Agostino, Veronica Maffia, Rita Colonna, Teresa Giuliano, Rosa Ferriero, Iolanda Boffa, Donna Palmer and 10 more

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In one paragraph

Article in The Journal of biological chemistry, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

20 authors.

Nunzia PastoreTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy; Department of Translational Medicine, Medical Genetics, Federico II University of Naples, Naples, Italy. Electronic address: pastore@tigem.it.
Sergio AttanasioTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Francesco AnnunziataTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Claudia D'AgostinoTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Veronica MaffiaTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Rita ColonnaTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Teresa GiulianoTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Rosa FerrieroTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Iolanda BoffaTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Donna PalmerDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.
Philip NgDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.
Rossella De CegliTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Massimo D'AgostinoDepartment of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy.
Florian RosenbergerDepartment of Medical Biochemistry and Biophysics, Division of Molecular Metabolism, Karolinska Institutet, Solna, Sweden.
Pavel StrnadMedical Clinic III, Gastroenterology, Metabolic Diseases, and Intensive Care, University Hospital RWTH Aachen, Aachen, Germany.
Joseph E ChambersCambridge Institute for Medical Research, Keith Peters Building, Cambridge Biomedical Campus, University of Cambridge, Cambridge, UK; Department of Medicine, University of Cambridge, Cambridge, UK.
Stefan J MarciniakCambridge Institute for Medical Research, Keith Peters Building, Cambridge Biomedical Campus, University of Cambridge, Cambridge, UK; Department of Medicine, University of Cambridge, Cambridge, UK.
Jeffrey TeckmanDepartment of Pediatrics and Department of Biochemistry and Molecular Biology, St Louis University School of Medicine, St Louis, Missouri, USA.
Pasquale PiccoloTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Nicola Brunetti-PierriTelethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy; Department of Translational Medicine, Medical Genetics, Federico II University of Naples, Naples, Italy; Scuola Superiore Meridionale (SSM, School of Advanced Studies), Genomics and Experimental Medicine Program, University of Naples Federico II, Naples, Italy. Electronic address: brunetti@tigem.it.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Liver disease in Alpha-1 antitrypsin deficiency (AATD) is caused by the toxic accumulation of mutant Z alpha-1 antitrypsin (Z-AAT) within the endoplasmic reticulum (ER) of hepatocytes. Livers from PiZ transgenic mice expressing the human Z-AAT and AATD patients who are homozygotes for the allele expressing Z-AAT were found to have increased p62/SQSTM1, a multifunctional protein involved in protein homeostasis. The goal of this study was to elucidate the involvement of p62/SQSTM1 in the formation of Z-AAT globules that are responsible for liver injury in AATD. In the present study, we found that p62/SQSTM1 decorated ubiquitin-positive, Periodic-Acid Shiff-diastase-resistant Z-AAT globules and interacted with Z-AAT at the ER-cytosol interface. Genetic ablation of p62/SQSTM1 in PiZ mice (PiZ;p62

Indexed as

AATDKEAP1NRF2p62/SQSTM1proteostasis

Identifiers

PMID42567518
PMCPMC13571921

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.