Evidence map›Paper›PMID 42370079›Full record

ReviewEULAR rheumatology open2026

Molecular mechanisms in rare proteasomopathies.

Sophie Wolfgramm, Flavia Llorente Alvarez, Franziska G Thiel, Martin Wendlandt, Elke Krüger

Abstract readReview
In one paragraph

Review in EULAR rheumatology open, 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

5 authors.

Sophie WolfgrammInstitute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, Greifswald, Germany.
Flavia Llorente AlvarezInstitute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, Greifswald, Germany.
Franziska G ThielInstitute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, Greifswald, Germany.
Martin WendlandtInstitute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, Greifswald, Germany.
Elke KrügerInstitute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, Greifswald, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Proteasomopathies comprise rare interferonopathy-related syndromes caused by genetic defects in proteasomal subunits or their assembly factors resulting in failed proteasome biogenesis and/or function. The concomitant proteasome impairment leads to imbalanced protein homeostasis by dysfunctional ubiquitin-mediated protein degradation. Two distinct clinical phenotypes have been characterised in proteasomopathies so far: (i) proteasome-associated autoinflammatory syndromes and (ii) proteasome-associated neurodevelopmental disorders. Despite these differences, both syndromes show molecular similarities with protein aggregation, activated stress responses, metabolic imbalance and dysregulated type I interferon signalling. Diagnostics and clinical management are complex even if genetic information is available. Here, we integrate the current knowledge of mammalian proteasome biogenesis with structural modelling of known proteasomopathy-causing variants and discuss the innovations of structural modelling to accelerate diagnosis.

Identifiers

PMID42370079
PMCPMC13292365

What Socratic holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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 Socratic graph.