The sigma-1 receptor at the crossroad of proteostasis, neurodegeneration, and autophagy

Sigma-1 Receptor Chaperones at the ER- Mitochondrion Interface Regulate Ca2+ Signaling and Cell Survival

Hayashi, T., & Su, T.-P. (2007) · Cell · doi:10.1016/j.cell.2007.08.036 · Cited by 1,826 · see citing works

Autophagosomes form at ER–mitochondria contact sites

Hamasaki, M., Furuta, N., Matsuda, A., Nezu, A., Yamamoto, A., Fujita, N., Oomori, H., Noda, T., Haraguchi, T., Hiraoka, Y., et al. (2013) · Nature · doi:10.1038/nature11910 · Cited by 1,747 · see citing works

Purification, molecular cloning, and expression of the mammalian sigma1-binding site.

Hanner, M., Moebius, F. F., Flandorfer, A., Knaus, H. G., Striessnig, J., Kempner, E. S., & Glossmann, H. H. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.15.8072 · Cited by 906 · see citing works

A mutation in sigma‐1 receptor causes juvenile amyotrophic lateral sclerosis

Al-Saif, A. M., Al-Mohanna, F. A., & Bohlega, S. (2011) · Annals of Neurology · doi:10.1002/ana.22534 · Cited by 430 · see citing works

Mitochondria‐associated membrane collapse is a common pathomechanism in SIGMAR1‐ and SOD1‐linked ALS

Watanabe, S., Ilieva, H. S., Tamada, H., Nomura, H., Komine, O., Endo, F., Jin, S., Mancías, P., Kiyama, H., & Yamanaka, K. (2016) · EMBO Molecular Medicine · doi:10.15252/emmm.201606403 · Cited by 231 · see citing works

Altered localization, abnormal modification and loss of function of Sigma receptor-1 in amyotrophic lateral sclerosis

Prause, J. U., Goswami, A., Katona, I., Roos, A., Schnizler, M. K., Bushuven, E., Dreier, A., Buchkremer, S., Johann, S., Beyer, C., et al. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt008 · Cited by 151 · see citing works

Sigma-1 receptor mediates cocaine-induced transcriptional regulation by recruiting chromatin-remodeling factors at the nuclear envelope

Tsai, S., Chuang, J., Tsai, M.-S., Wang, X.-F., Xi, Z., Hung, J., Chang, W., Bonci, A., & Su, T. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1518894112 · Cited by 115 · see citing works

The ALS-linked E102Q mutation in Sigma receptor-1 leads to ER stress-mediated defects in protein homeostasis and dysregulation of RNA-binding proteins

Dreser, A., Vollrath, J. T., Sechi, A. S., Johann, S., Roos, A., Yamoah, A., Katona, I., Bohlega, S., Wiemuth, D., Tian, Y., et al. (2017) · Cell Death and Differentiation · doi:10.1038/cdd.2017.88 · Cited by 99 · see citing works

Targeting the Sigma-1 Receptor via Pridopidine Ameliorates Central Features of ALS Pathology in a SOD1G93A Model

Ionescu, A., Gradus, T., Altman, T., Maimon, R., Avraham, N. S., Geva, M., Hayden, M. R., & Perlson, E. (2019) · Cell Death and Disease · doi:10.1038/s41419-019-1451-2 · Cited by 92 · see citing works

SIGMAR1/Sigma-1 receptor ablation impairs autophagosome clearance

Yang, H. M., Shen, H., Li, J., & Guo, L.-W. (2019) · Autophagy · doi:10.1080/15548627.2019.1586248 · Cited by 85 · see citing works

Sigma-1 Receptor Activation Induces Autophagy and Increases Proteostasis Capacity In Vitro and In Vivo

Christ, M., Huesmann, H., Nagel, H., Kern, A., & Behl, C. (2019) · Cells · doi:10.3390/cells8030211 · Cited by 74 · see citing works

ANAVEX®2-73 (blarcamesine), a Sigma-1 receptor agonist, ameliorates neurologic impairments in a mouse model of Rett syndrome

Kaufmann, W. E., Sprouse, J., Rebowe, N., Hanania, T., Klamer, D., & Missling, C. U. (2019) · Pharmacology Biochemistry and Behavior · doi:10.1016/j.pbb.2019.172796 · Cited by 54 · see citing works

An Update on Psychopharmacological Treatment of Autism Spectrum Disorder

Aishworiya, R., Valica, T., Hagerman, R., & Restrepo, B. (2022) · Neurotherapeutics · doi:10.1007/s13311-022-01183-1 · Cited by 233 · see citing works

Repurposing Sigma-1 Receptor Ligands for COVID-19 Therapy?

Vela, J. M. (2020) · Frontiers in Pharmacology · doi:10.3389/fphar.2020.582310 · Cited by 97 · see citing works

Targeting Sigma Receptors for the Treatment of Neurodegenerative and Neurodevelopmental Disorders

Malar, D. S., Thitilertdecha, P., Ruckvongacheep, K. S., Brimson, S., Tencomnao, T., & Brimson, J. M. (2023) · CNS Drugs · doi:10.1007/s40263-023-01007-6 · Cited by 74 · see citing works

Abnormalities of synaptic mitochondria in autism spectrum disorder and related neurodevelopmental disorders

Rojas-Charry, L., Nardi, L., Methner, A., & Schmeißer, M. J. (2020) · Journal of Molecular Medicine · doi:10.1007/s00109-020-02018-2 · Cited by 59 · see citing works

Focusing on the Unfolded Protein Response and Autophagy Related Pathways to Reposition Common Approved Drugs against COVID-19Preprint

Nabirotchkin, S., Peluffo, A. E., Bouaziz, J., & Cohen, D. (2020) · Preprints.org · doi:10.20944/preprints202003.0302.v1 · Cited by 49 · see citing works

Emerging potential of cannabidiol in reversing proteinopathies

Dash, R., Ali, M. C., Jahan, I., Munni, Y. A., Mitra, S., Hannan, M. A., Timalsina, B., Oktaviani, D. F., Choi, H. J., & Moon, I. S. (2020) · Ageing Research Reviews · doi:10.1016/j.arr.2020.101209 · Cited by 49 · see citing works

Stimulation of Sigma‐1 Receptor Protects against Cardiac Fibrosis by Alleviating IRE1 Pathway and Autophagy Impairment

Qu, J., Li, M., Li, D., Xin, Y., Li, J., Song, L., Wu, W., & Liu, X. (2021) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2021/8836818 · Cited by 44 · see citing works

Mitochondrial Modulations, Autophagy Pathways Shifts in Viral Infections: Consequences of COVID-19

Singh, S. P., Amar, S., Gehlot, P., Patra, S. K., Kanwar, N., & Kanwal, A. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22158180 · Cited by 34 · see citing works

Multi-Target Directed Ligands (MTDLs) Binding the σ1 Receptor as Promising Therapeutics: State of the Art and Perspectives

Abatematteo, F. S., Niso, M., Contino, M., Leopoldo, M., & Abate, C. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22126359 · Cited by 29 · see citing works

Novel Therapeutic Target for Prevention of Neurodegenerative Diseases: Modulation of Neuroinflammation with Sig-1R Ligands

Bogár, F., Fülöp, L., & Penke, B. (2022) · Biomolecules · doi:10.3390/biom12030363 · Cited by 27 · see citing works

Chaperone-Dependent Mechanisms as a Pharmacological Target for Neuroprotection

Воронин, М. В., Абрамова, Е. В., Verbovaya, E. R., Vakhitova, Y. V., & Середенин, С. Б. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24010823 · Cited by 27 · see citing works

Central role of Sigma-1 receptor in ochratoxin A-induced ferroptosis

Chen, W., Han, L., Yang, R., Wang, H., Yao, S., Deng, H., Liu, S., Zhou, Y., & Shen, X. L. (2024) · Archives of Toxicology · doi:10.1007/s00204-024-03805-3 · Cited by 21 · see citing works

The role of autophagy in the pathogenesis and treatment of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

Beckers, J., & Damme, P. V. (2025) · Autophagy Reports · doi:10.1080/27694127.2025.2474796 · Cited by 16 · see citing works

Ochratoxin A induces mitochondrial apoptosis and ferroptosis by inhibiting sigma-1 receptor to disrupt redox and cholesterol homeostasis

Yao, S., Chen, W., Wang, H., Yang, R., Zhou, Y., Liu, S., & Shen, X. L. (2024) · Food Science and Human Wellness · doi:10.26599/fshw.2024.9250372 · Cited by 15 · see citing works

Sigma-1 Receptor in Retina: Neuroprotective Effects and Potential Mechanisms

Xu, Z., Lei, Y., Qin, H., Zhang, S., Li, P., & Yao, K. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23147572 · Cited by 14 · see citing works

The Interplay Between Autophagy and RNA Homeostasis: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

Houghton, O. H., Mizielinska, S., & Gómez‐Suaga, P. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.838402 · Cited by 12 · see citing works

An Update on Psychopharmacological Treatment of Autism Spectrum Disorder

Aishworiya, R., Valica, T., Hagerman, R. J., & Restrepo, B. (2024) · FOCUS The Journal of Lifelong Learning in Psychiatry · doi:10.1176/appi.focus.24022006 · Cited by 15 · see citing works

Novel High Affinity Sigma-1 Receptor Ligands from Minimal Ensemble Docking-Based Virtual Screening

Dvorácskó, S., Lázár, L., Fülöp, F., Palkó, M., Zalán, Z., Penke, B., Fülöp, L., Tömböly, C., & Bogár, F. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22158112 · Cited by 12 · see citing works

Sigma-1 receptor ablation impedes adipocyte-like differentiation of mouse embryonic fibroblasts

Yang, H. M., Shen, H., Li, J., Stanford, K. I., & Guo, L. (2020) · Cellular Signalling · doi:10.1016/j.cellsig.2020.109732 · Cited by 8 · see citing works

Concentration–QTc Relationship from a Single Ascending Dose Study of ANAVEX3‐71, a Novel Sigma‐1 Receptor and Allosteric M1 Muscarinic Receptor Agonist in Development for the Treatment of Frontotemporal Dementia, Schizophrenia, and Alzheimer's Disease

Fadiran, E. O., Hammond, E. R., Tran, J., Xue, H., Chen, J., Kaufmann, W. E., Missling, C. U., & Darpö, B. (2023) · Clinical Pharmacology in Drug Development · doi:10.1002/cpdd.1303 · Cited by 7 · see citing works

Conserved LIR-specific interaction of Sigma-1 receptor and GABARAP

Baeken, M. W., Christ, M., Schmitt, D., Trein, W., Nagel, H., Clement, A. M., Körschgen, H., & Behl, C. (2025) · iScience · doi:10.1016/j.isci.2025.113287 · Cited by 6 · see citing works

Human Cell Organelles in SARS-CoV-2 Infection: An Up-to-Date Overview

Gorący, A., Rosik, J., Szostak, B., Ustianowski, Ł., Ustianowska, K., & Gorący, J. (2022) · Viruses · doi:10.3390/v14051092 · Cited by 5 · see citing works

Brain cell signaling abnormalities are detected in blood in a murine model of Fragile X syndrome and corrected by Sigma‐1 receptor agonist Blarcamesine

Cogram, P., Deacon, R. M. J., Klamer, D., Rebowe, N., Sprouse, J. S., Reyes, S. T., Missling, C. U., & Kaufmann, W. E. (2022) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62853 · Cited by 5 · see citing works

Mitochondria-associated niches in health and disease

Milani, M., Pihán, P., & Hetz, C. A. (2022) · Journal of Cell Science · doi:10.1242/jcs.259634 · Cited by 4 · see citing works

The sigma-1 receptor as a neurohomeostatic decision hub for GABARAP-mediated receptor trafficking and macroautophagy

Baeken, M. W., Bekbulat, F., Körschgen, H., Clement, A. M., & Behl, C. (2025) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2025.1673249 · Cited by 4 · see citing works

Targeting Neuroinflammation in Depression: The Integrative Role of Sigma‐1 Receptor Modulation

Wang, J., Yang, D., Li, Y., & Ren, P. (2026) · Journal of Neurochemistry · doi:10.1111/jnc.70490 · Cited by 3 · see citing works

Roles of Cannabidiol in Reversing ProteinopathiesPreprint

Dash, R., Ali, M. C., Jahan, I., Munni, Y. A., Mitra, S., Hannan, M. A., Timalsina, B., Oktaviani, D. F., Choi, H. J., & Moon, I. S. (2020) · Preprints.org · doi:10.20944/preprints202005.0340.v1 · Cited by 3 · see citing works

Hyperphagia after Magnetic Resonance‐Guided Focused Ultrasound Thalamotomy for Essential Tremor: Role of the Zona Incerta

Hanada, T., Yamada, K., Horisawa, S., Higashi, T., Makiuchi, T., Tokimura, Y., & Hanaya, R. (2025) · Movement Disorders Clinical Practice · doi:10.1002/mdc3.70416 · Cited by 1 · see citing works

Biophysical Evaluation and In Vitro Controlled Release of Two Isomeric Adamantane Phenylalkylamines with Antiproliferative/Anticancer and Analgesic Activity

Vlachou, M., Foscolos, A., Siamidi, A., Syriopoulou, A., Georgiou, N., Dedeloudi, A., Tsiailanis, A. D., Tzakos, A. G., Mavromoustakos, T., & Papanastasiou, I. P. (2021) · Molecules · doi:10.3390/molecules27010007 · Cited by 1 · see citing works

Oral Blarcamesine Phase IIb/III Trial Confirms Identified Precision Medicine Patient Population – Significant Broad Clinical and Quality of Life Improvements for Early Alzheimer’s Disease PatientsPreprint

Macfarlane, S., Grimmer, T., Teo, K. G., O'Brien, T. J., Woodward, M., Grunfeld, J. A., Mander, A. G., Brew, B. J., Morris, P., Short, C., et al. (2025) · medRxiv · doi:10.1101/2025.09.27.25336656 · Cited by 1 · see citing works

Anaesthesia drugs, SARS-CoV-2, and the sigma-1 receptor: a complex affair. Comment on Br J Anaesth 2021; 127: e32–4

Jain, A., Lamperti, M., Doyle, D. J., & Lobo, F. A. (2021) · British Journal of Anaesthesia · doi:10.1016/j.bja.2021.09.008

Alternate mechanisms of SARS-CoV-2–induced analgesia and additional pathological significance of SARS-CoV-2 spike protein interaction with vascular endothelial growth factor-A/neuropilin-1 receptor signaling

Jain, A., Tolba, R., Salti, A., & Lamperti, M. (2021) · Pain · doi:10.1097/j.pain.0000000000002447

Integrated control of cancer stemness by σ1 receptor in advanced prostate cancer

Civenni, G., Sandrini, G., Merulla, J., Musumeci, C., Federici, E., Vallerga, A., Kokanovic, A., Mosole, S., Shinde, D. A., Sorrenti, E., et al. (2025) · Oncogene · doi:10.1038/s41388-025-03541-7

Interplay Between Autophagy, Cellular Senescence, and Brain Aging: Neuroprotective Implications of Intermittent Fasting

Singh, I., Bhat, S., Tamatta, R., & Singh, A. K. (2026) · Cellular and Molecular Neurobiology · doi:10.1007/s10571-026-01709-7 · Cited by 1 · see citing works

„Wenn Sie die Art und Weise ändern, wie Sie Dinge betrachten, ändern sich die Dinge, die Sie betrachten“

Behl, C. (2026) · Springer eBooks · doi:10.1007/978-3-662-72210-7_21

From Structurally Diverse Sigma-1 (σ1) Receptor Reference Ligands to Live-Cell Probes: Fluorescent Phenoxyalkylpiperidines for the Detection of σ1 Receptor

Musillo, G. R., Abatematteo, F. S., Stiller, B., Majellaro, M., Gioé-Gallo, C., Ortigueira, S., Niso, M., Liturri, A., Perrone, M. G., Vavers, E., et al. (2026) · Journal of Medicinal Chemistry · doi:10.1021/acs.jmedchem.6c02423

Sigma-1 Receptor Activation Induces Autophagy and Increases Proteostasis Capacity In Vitro and In Vivo

Christ, M., Huesmann, H., Nagel, H., Kern, A., & Behl, C. (2019) · Cells · doi:10.3390/cells8030211 · Cited by 74 · see citing works

The involvement of the sigma-1 receptor in neurodegeneration and neurorestoration

Ruscher, K., & Wieloch, T. W. (2014) · Journal of Pharmacological Sciences · doi:10.1016/j.jphs.2014.11.011 · Cited by 107 · see citing works

Sigma-1 Receptors and Neurodegenerative Diseases: Towards a Hypothesis of Sigma-1 Receptors as Amplifiers of Neurodegeneration and Neuroprotection

Nguyen, L., Lucke-Wold, B. P., Mookerjee, S. A., Kaushal, N., & Matsumoto, R. R. (2017) · Advances in experimental medicine and biology · doi:10.1007/978-3-319-50174-1_10 · Cited by 119 · see citing works

SIGMAR1 Confers Innate Resilience against Neurodegeneration

Couly, S., Yasui, Y., & Su, T. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24097767 · Cited by 29 · see citing works

Emerging Benefits: Pathophysiological Functions and Target Drugs of the Sigma-1 Receptor in Neurodegenerative Diseases

Wu, N., Ye, Y., Wan, B.-B., Yu, Y.-D., Liu, C., & Chen, Q. (2021) · Molecular Neurobiology · doi:10.1007/s12035-021-02524-5 · Cited by 55 · see citing works