4.8 Article

Nucleoporin POM121 signals TFEB-mediated autophagy via activation of SIGMAR1/sigma-1 receptor chaperone by pridopidine

相关参考文献

注意:仅列出部分参考文献,下载原文获取全部文献信息。
Review Pharmacology & Pharmacy

Bi-phasic dose response in the preclinical and clinical developments of sigma-1 receptor ligands for the treatment of neurodegenerative disorders

Tangui Maurice

Summary: The sigma-1 receptor (S1R) is a conserved protein with high therapeutic potential for neurodegenerative diseases, especially with drugs like pridopidine. S1R agonists modulate inter-organelle communication, activate intracellular survival pathways, and exhibit a bi-phasic dose-response effect. More research is needed to fully understand the cellular nature of this chaperone protein for the development of promising molecules.

EXPERT OPINION ON DRUG DISCOVERY (2021)

Article Neurosciences

Genomic Action of Sigma-1 Receptor Chaperone Relates to Neuropathic Pain

Shao-Ming Wang et al.

Summary: The study revealed that Sig-1R indirectly contributes to neuropathic pain by decreasing the protein level of Cav2.2, thereby increasing neuronal excitability in dorsal root ganglia. Furthermore, Sig-1R was found to interact with other proteins in the nucleus, affecting complex signaling pathways within neurons.

MOLECULAR NEUROBIOLOGY (2021)

Article Multidisciplinary Sciences

Mechanism of the small ATP-independent chaperone Spy is substrate specific

Rishav Mitra et al.

Summary: ATP-independent chaperone Spy demonstrates substrate-specific behavior when interacting with the complex client apoflavodoxin, tightly binding to partially unfolded states to convert into a pure holdase.

NATURE COMMUNICATIONS (2021)

Article Biochemistry & Molecular Biology

Sigma-1 Receptor (S1R) Interaction with Cholesterol: Mechanisms of S1R Activation and Its Role in Neurodegenerative Diseases

Vladimir Zhemkov et al.

Summary: The study investigates the interaction between the sigma-1 receptor (S1R) and cholesterol, showing that S1R association with cholesterol induces the formation of S1R clusters, leading to cholesterol-enriched microdomains that may attract and retain various secreted and signaling proteins.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

Article Biochemistry & Molecular Biology

Pridopidine reduces mutant huntingtin-induced endoplasmic reticulum stress by modulation of the Sigma-1 receptor

Marina Shenkman et al.

Summary: Pridopidine can significantly alleviate mHtt-induced ER stress, reduce markers of the three UPR branches with the strongest effect on the PERK branch. This effect is S1R-dependent and may be achieved by increasing insoluble S1R and stabilizing activated S1R oligomers.

JOURNAL OF NEUROCHEMISTRY (2021)

Article Clinical Neurology

The Sigma-1 Receptor Mediates Pridopidine Rescue of Mitochondrial Function in Huntington Disease Models

Luana Naia et al.

Summary: Pridopidine is a selective Sigma-1 receptor agonist being developed for Huntington disease and amyotrophic lateral sclerosis. It enhances mitochondrial function by improving activity, elongation, and motility, leading to neuroprotective effects in HD models. Pridopidine also reduces ROS levels and restores antioxidant response in various HD models, showing potential therapeutic benefits.

NEUROTHERAPEUTICS (2021)

Article Biology

The role of sigma 1 receptor in organization of endoplasmic reticulum signaling microdomains

Vladimir Zhemkov et al.

Summary: The findings suggest that S1R interacts with cholesterol to form cholesterol-enriched microdomains in the ER membrane, impacting its biological activity in cells. Additionally, S1R agonists disrupt S1R clusters, indicating a link between their biological activity and remodeling of ER membrane microdomains.
Article Cell Biology

Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS

Alyssa N. Coyne et al.

Summary: Alterations in the components and function of the nuclear pore complex have been implicated in the pathogenesis of genetic forms of neurodegeneration, and CHMP7 has been identified as a critical mediator of NPC quality control. Increasing evidence suggests that altered CHMP7-mediated Nup homeostasis may play a prominent role in the development of familial and sporadic ALS, highlighting CHMP7 as a potential therapeutic target.

SCIENCE TRANSLATIONAL MEDICINE (2021)

Article Clinical Neurology

Sigma-1 (sigma(1)) receptor activity is necessary for physiological brain plasticity in mice

Lucie Crouzier et al.

EUROPEAN NEUROPSYCHOPHARMACOLOGY (2020)

Article Neurosciences

Effects of Pridopidine on Functional Capacity in Early-Stage Participants from the PRIDE-HD Study

Andrew McGarry et al.

JOURNAL OF HUNTINGTONS DISEASE (2020)

Article Neurosciences

Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor

Chelsy R. Eddings et al.

NEUROBIOLOGY OF DISEASE (2019)

Article Biochemistry & Molecular Biology

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

Ke Zhang et al.

Review Biochemistry & Molecular Biology

The complex relationship between TFEB transcription factor phosphorylation and subcellular localization

Rosa Puertollano et al.

EMBO JOURNAL (2018)

Editorial Material Neurosciences

Nuclear pores: the gate to neurodegeneration

Nan Li et al.

NATURE NEUROSCIENCE (2018)

Article Multidisciplinary Sciences

A TFEB nuclear export signal integrates amino acid supply and glucose availability

Linxin Li et al.

NATURE COMMUNICATIONS (2018)

Article Biochemistry & Molecular Biology

Nuclear Pores Promote Lethal Prostate Cancer by Increasing POM121-Driven E2F1, MYC, and AR Nuclear Import

Veronica Rodriguez-Bravo et al.

Review Ophthalmology

Sigma 1 receptor: A novel therapeutic target in retinal disease

Sylvia B. Smith et al.

PROGRESS IN RETINAL AND EYE RESEARCH (2018)

Article Multidisciplinary Sciences

The sigma-1 receptor modulates methamphetamine dysregulation of dopamine neurotransmission

Danielle O. Sambo et al.

NATURE COMMUNICATIONS (2017)

Article Medicine, Research & Experimental

Early pridopidine treatment improves behavioral and transcriptional deficits in YAC128 Huntington disease mice

Marta Garcia-Miralles et al.

JCI INSIGHT (2017)

Article Biochemistry & Molecular Biology

Pridopidine activates neuroprotective pathways impaired in Huntington Disease

Michal Geva et al.

HUMAN MOLECULAR GENETICS (2016)

Article Multidisciplinary Sciences

Crystal structure of the human σ1 receptor

Hayden R. Schmidt et al.

NATURE (2016)

Article Biochemistry & Molecular Biology

σ1 receptor ligands control a switch between passive and active threat responses

Andrew J. Rennekamp et al.

NATURE CHEMICAL BIOLOGY (2016)

Article Biochemistry & Molecular Biology

Toward the atomic structure of the nuclear pore complex: when top down meets bottom up

Andre Hoelz et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2016)

Article Multidisciplinary Sciences

Activation of the molecular chaperone, sigma 1 receptor, preserves cone function in a murine model of inherited retinal degeneration

Jing Wang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)

Review Pharmacology & Pharmacy

The Sigma-1 Receptor as a Pluripotent Modulator in Living Systems

Tsung-Ping Su et al.

TRENDS IN PHARMACOLOGICAL SCIENCES (2016)

Article Biochemistry & Molecular Biology

Toward the atomic structure of the nuclear pore complex: when top down meets bottom up

Andre Hoelz et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2016)

Article Multidisciplinary Sciences

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

Ke Zhang et al.

NATURE (2015)

Article Multidisciplinary Sciences

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

Brian D. Freibaum et al.

NATURE (2015)

Article Multidisciplinary Sciences

Transcriptional control of autophagy-lysosome function drives pancreatic cancer metabolism

RushikaM. Perera et al.

NATURE (2015)

Article Multidisciplinary Sciences

Subcellular Localization of the Sigma-1 Receptor in Retinal Neurons - an Electron Microscopy Study

Timur A. Mavlyutov et al.

SCIENTIFIC REPORTS (2015)

Article Biochemistry & Molecular Biology

The Oligomeric States of the Purified Sigma-1 Receptor Are Stabilized by Ligands

Katarzyna A. Gromek et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2014)

Article Multidisciplinary Sciences

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

Aaron R. Haeusler et al.

NATURE (2014)

Article Clinical Neurology

Sigma-1R Agonist Improves Motor Function and Motoneuron Survival in ALS Mice

Renzo Mancuso et al.

NEUROTHERAPEUTICS (2012)

Article Clinical Neurology

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

Amr Al-Saif et al.

ANNALS OF NEUROLOGY (2011)

Article Multidisciplinary Sciences

TFEB Links Autophagy to Lysosomal Biogenesis

Carmine Settembre et al.

SCIENCE (2011)

Article Multidisciplinary Sciences

The Hallucinogen N,N-Dimethyltryptamine (DMT) Is an Endogenous Sigma-1 Receptor Regulator

Dominique Fontanilla et al.

SCIENCE (2009)

Article Multidisciplinary Sciences

A Gene Network Regulating Lysosomal Biogenesis and Function

Marco Sardiello et al.

SCIENCE (2009)

Article Pharmacology & Pharmacy

Clinical trials with sigma ligands

HP Volz et al.

PHARMACOPSYCHIATRY (2004)