Pridopidine Protects ALS Patient-Derived Neural Progenitor Cells via Sigma-1 Receptor Activation.

May Meltzer, Maya Shefler Zamir, Noam Tzuri, Andrew M Tan, Michal Geva, Michael R Hayden, Rachel G Lichtenstein

Abstract read

In one paragraph

Article in International journal of molecular sciences, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it

0cells of the map it votes in

1citing 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

1 citing paper in PubMed.

Pridopidine Mediated Sigma-1 Receptor Activation and Therapeutic Implications in Neurodegenerative Diseases.Neurology international · 2026
Review

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

7 authors.

May MeltzerPrilenia Therapeutics B.V., 1411 DC Naarden, The Netherlands.

Maya Shefler ZamirAvram and Stella Goren-Goldstein Department of Biotechnology Engineering, Faculty of Engineering, Ben-Gurion University of the Negev, P.O. Box 653, Beer-Sheva 84105, Israel.ORCID 0009-0004-4828-0811

Noam TzuriAvram and Stella Goren-Goldstein Department of Biotechnology Engineering, Faculty of Engineering, Ben-Gurion University of the Negev, P.O. Box 653, Beer-Sheva 84105, Israel.

Andrew M TanPrilenia Therapeutics B.V., 1411 DC Naarden, The Netherlands.ORCID 0000-0002-0099-8814

Michal GevaPrilenia Therapeutics B.V., 1411 DC Naarden, The Netherlands.

Michael R HaydenPrilenia Therapeutics B.V., 1411 DC Naarden, The Netherlands.

Rachel G LichtensteinAvram and Stella Goren-Goldstein Department of Biotechnology Engineering, Faculty of Engineering, Ben-Gurion University of the Negev, P.O. Box 653, Beer-Sheva 84105, Israel.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

The sigma-1 receptor (S1R) is an endoplasmic reticulum (ER)-resident protein enriched at the mitochondria-associated ER membranes (MAMs) that supports ER homeostasis, preserves mitochondrial function, and enhances cell survival under stress. Disruptions of MAM integrity and prolonged ER stress are well-recognized pathological features of amyotrophic lateral sclerosis (ALS), contributing to motor neuron dysfunction and degeneration. In this study, we evaluated the protective effects of pridopidine, a highly selective and potent S1R agonist currently in clinical development for Huntington's disease (HD) and ALS, using neural progenitor cells (NPCs) derived from induced pluripotent stem cells (iPSCs) from a patient with sporadic ALS. Exposure of ALS NPCs to the ER stressor tunicamycin increased the ER stress markers binding immunoglobulin protein (BiP) and C/EBP homologous protein (CHOP), disrupted mitochondrial membrane potential, upregulated expression of the mitochondrial apoptotic marker,

Indexed as

Amyotrophic Lateral SclerosisNeural Stem CellsPiperidinesReceptors, sigmaApoptosisCell SurvivalEndoplasmic Reticulum StressHumansInduced Pluripotent Stem CellsMembrane Potential, MitochondrialMitochondriaNeuroprotective AgentsSigma-1 ReceptorTranscription Factor CHOPTunicamycinNeuroprotective AgentsPiperidinespridopidineReceptors, sigmaSigma-1 ReceptorTranscription Factor CHOPTunicamycinamyotrophic lateral sclerosisendoplasmic reticulum stressiPSC-derived neural progenitor cellsmitochondria-associated membranesmitochondrial membrane potentialpridopidineSigma-1 receptor

Identifiers

PMID42074133

DOI10.3390/ijms27083489

PMCPMC13116552

What Socratic holds

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LicenceCC BY

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Registered trials

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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 Socratic graph.