Evidence map›Paper›PMID 41384450›Full record

ArticleDisease models & mechanisms2026

An astrocytic cellular model of Lafora disease to study polyglucosan accumulation and inflammation.

Mireia Moreno-Estellés, Angela Campos-Rodríguez, Rosa Viana, Laura Baños-Carrión, Marta Albuixech, Maria A García-Gimeno, Matthew S Gentry, Pascual Sanz

Abstract read
In one paragraph

Article in Disease models & mechanisms, 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

8 authors.

Mireia Moreno-EstellésInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.ORCID 0000-0001-8759-8794
Angela Campos-RodríguezInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.
Rosa VianaInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.
Laura Baños-CarriónInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.
Marta AlbuixechInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.
Maria A García-GimenoDepartment of Biotechnology, Universitat Politécnica de València, 46022 Valencia, Spain.
Matthew S GentryDepartment of Biochemistry and Molecular Biology, University of Florida College of Medicine, Gainesville, FL 32610, USA.
Pascual SanzInstituto de Biomedicina de Valencia, Nutrient Signaling Unit, CSIC, Jaime Roig 11, 46010 Valencia, Spain.ORCID 0000-0002-2399-4103

Funding

Suppressing glycogen storage with small molecule inhibitors as a therapeutic approach to Lafora DiseaseP01NS097197 · NINDS · UNIVERSITY OF KENTUCKY · PI GAO, TIANYAN · 2016 to 2021
$9.0M
Brain Glycogen-Metabolism,Mechanisms, and Therapeutic PotentialR35NS116824 · NINDS · UNIVERSITY OF KENTUCKY · PI Matthew S. Gentry · 2020 to 2026
$8.2M
Centro de Investigación Biomédica en Red de Enfermedades Raras ACCI2023-03-742CIBERER ACCI2023Consejo Superior de Investigaciones CientíficasConselleria de Cultura, Educacion y Ciencia, Generalitat Valenciana CIPROM2022/42Generalitat Valenciana CIPROM2022/42Ministerio de Ciencia e Innovacion PID2023-14803OB-I00Ministerio de Ciencia e Innovación PID2023-1481023OB-I00NINDS NIH HHS P01 NS097197NINDS NIH HHS R35 NS116824
6 · The paper itself

Abstract

Lafora disease (LD) is a devastating form of progressive myoclonus epilepsy characterized by the accumulation of insoluble forms of glycogen [polyglucosan bodies (PGBs)] in the brain and peripheral tissues. It has been proposed that the accumulation of PGBs is pathogenic. Several mouse models of LD have been generated to study the relationship between PGBs and the pathophysiology of LD. However, the use of LD mice is difficult and time consuming; thus, more amenable cellular systems would be desirable. We recently described a cellular model based on the culture of primary postnatal astrocytes from LD mice that are able to accumulate small PGBs. In this study, we extended this astrocytic model by maturing the astrocytes for longer times. These more mature astrocyte cultures accumulated larger and granular PGBs, which have similar properties to the ones present in the hippocampus of Nhlrc1-/- (Epm2b-/-) mice. Importantly, this model expresses inflammatory mediators related to LD pathophysiology. This astrocytic model could be used to better understand the formation of the PGBs and also to define how the accumulation of PGBs activates the expression of inflammatory mediators.

Indexed as

AstrocytesGlucansInflammationLafora DiseaseModels, BiologicalAnimalsCells, CulturedDisease Models, AnimalGlycogenHippocampusInflammation MediatorsMiceMice, Inbred C57BLUbiquitin-Protein LigasesGlucansGlycogenInflammation MediatorsNHLRC1 protein, mousepolyglucosanUbiquitin-Protein LigasesAstrocyte modelInflammationLafora diseasePolyglucosan

Identifiers

PMID41384450
PMCPMC12919960

What OpenQuestion 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 OpenQuestion graph.