Sigma-1 Receptor Stimulation Rescues FTD/ALS Mutant TDP43-Induced Disruption of the VAPB-PTPIP51 ER-Mitochondria Tethering Proteins via Inhibition of GSK3β.

doi:10.3390/cells15171536

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Sigma-1 Receptor Stimulation Rescues FTD/ALS Mutant TDP43-Induced Disruption of the VAPB-PTPIP51 ER-Mitochondria Tethering Proteins via Inhibition of GSK3β.

Cells - 26 Aug 2026

Blair Kerry, Gosset Philippe, Martinez-Serra Raquel, Mórotz Gábor M, Martín-Guerrero Sandra M, Gomez-Suaga Patricia, Atherton Joseph, Mitchell Jacqueline C, Noble Wendy, Miller Christopher C J, Markovinovic Andrea

Abstract excerpt

Signalling between the ER and mitochondria regulates a number of key cellular functions that are damaged in frontotemporal dementia and related amyotrophic lateral sclerosis (FTD/ALS). This signalling involves close physical contacts between the two organelles that are mediated by the VAPB-PTPIP51 ER-mitochondria "tethering" proteins. A number of studies have shown that mutant genes which cause familial FTD/ALS...

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Topics

Receptors, sigma
Sigma-1 Receptor
Glycogen Synthase Kinase 3 beta
Humans
DNA-Binding Proteins

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Endoplasmic Reticulum

Mitochondria

Amyotrophic Lateral Sclerosis

Mutation

Frontotemporal Dementia

Mitochondrial Proteins

Animals

Protein Binding

Mitochondria Associated Membranes

Calcium

Source

Open in PubMed Open full text

Identifiers

PMID: 42738830
DOI: 10.3390/cells15171536
PMCID: PMC13565437

Genes

VAPB

Provenance

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Sigma-1 Receptor Stimulation Rescues FTD/ALS Mutant TDP43-Induced Disruption of the VAPB-PTPIP51 ER-Mitochondria Tethering Proteins via Inhibition of GSK3β.PMID 42738830Read ↗

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