SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides

SARM1 is a key regulator of a conserved program of axon degeneration increasingly linked to human neurodegenerative diseases. Pathological SARM1 activation causes rapid NAD consumption, disrupting cellular homeostasis and leading to axon degeneration. In this study, we develop antisense oligonucleot...

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Main Authors: Andrea Loreto, Kaitlyn M.L. Cramb, Lucy A. McDermott, Christina Antoniou, Ilenia Cirilli, Maria Claudia Caiazza, Elisa Merlini, Peter Arthur-Farraj, W. Daniel du Preez, Elliot D. Mock, Hien T. Zhao, David L. Bennett, Giuseppe Orsomando, Michael P. Coleman, Richard Wade-Martins
Format: Article
Language:English
Published: Elsevier 2025-09-01
Series:Neurobiology of Disease
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Online Access:http://www.sciencedirect.com/science/article/pii/S0969996125002025
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author Andrea Loreto
Kaitlyn M.L. Cramb
Lucy A. McDermott
Christina Antoniou
Ilenia Cirilli
Maria Claudia Caiazza
Elisa Merlini
Peter Arthur-Farraj
W. Daniel du Preez
Elliot D. Mock
Hien T. Zhao
David L. Bennett
Giuseppe Orsomando
Michael P. Coleman
Richard Wade-Martins
author_facet Andrea Loreto
Kaitlyn M.L. Cramb
Lucy A. McDermott
Christina Antoniou
Ilenia Cirilli
Maria Claudia Caiazza
Elisa Merlini
Peter Arthur-Farraj
W. Daniel du Preez
Elliot D. Mock
Hien T. Zhao
David L. Bennett
Giuseppe Orsomando
Michael P. Coleman
Richard Wade-Martins
author_sort Andrea Loreto
collection DOAJ
description SARM1 is a key regulator of a conserved program of axon degeneration increasingly linked to human neurodegenerative diseases. Pathological SARM1 activation causes rapid NAD consumption, disrupting cellular homeostasis and leading to axon degeneration. In this study, we develop antisense oligonucleotides (ASOs) targeting human SARM1, demonstrating robust neuroprotection against morphological, metabolic, and mitochondrial impairment in human iPSC-derived dopamine neurons induced by the lethal neurotoxin vacor, a potent SARM1 activator. Furthermore, our findings reveal that axon fragmentation can be prevented, and mitochondrial dysfunction reversed using the NAD precursor nicotinamide, a form of vitamin B3, even after SARM1 activation has occurred, when neurons are already unhealthy. This research identifies ASOs as a promising therapeutic strategy to block SARM1, and provides an extensive characterisation and further mechanistic insights that demonstrate the reversibility of SARM1 toxicity in human neurons. It also identifies the SARM1 activator vacor as a specific and reversible neuroablative agent in human neurons.
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spelling doaj-art-e7b0c5154f0f43d38d085e503fc5ade52025-08-20T03:13:40ZengElsevierNeurobiology of Disease1095-953X2025-09-0121310698610.1016/j.nbd.2025.106986SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotidesAndrea Loreto0Kaitlyn M.L. Cramb1Lucy A. McDermott2Christina Antoniou3Ilenia Cirilli4Maria Claudia Caiazza5Elisa Merlini6Peter Arthur-Farraj7W. Daniel du Preez8Elliot D. Mock9Hien T. Zhao10David L. Bennett11Giuseppe Orsomando12Michael P. Coleman13Richard Wade-Martins14Neuroscience, School of Medical Sciences, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Save Sight Institute, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Charles Perkins Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Oxford Parkinson's Disease Centre and Department of Physiology, Anatomy & Genetics, Kavli Institute for Nanoscience Discovery, University of Oxford, Dorothy Crowfoot Hodgkin Building, South Parks Road, Oxford OX1 3QU, UK; John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, CB2 0PY Cambridge, UK; Corresponding author at: Neuroscience, School of Medical Sciences, Faculty of Medicine and Health, University of Sydney, Sydney, Australia.Oxford Parkinson's Disease Centre and Department of Physiology, Anatomy & Genetics, Kavli Institute for Nanoscience Discovery, University of Oxford, Dorothy Crowfoot Hodgkin Building, South Parks Road, Oxford OX1 3QU, UK; Corresponding authors.Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UKJohn van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, CB2 0PY Cambridge, UKDepartment of Clinical Sciences (DISCO), Section of Biochemistry, Polytechnic University of Marche, Via Ranieri 67, Ancona 60131, ItalyOxford Parkinson's Disease Centre and Department of Physiology, Anatomy & Genetics, Kavli Institute for Nanoscience Discovery, University of Oxford, Dorothy Crowfoot Hodgkin Building, South Parks Road, Oxford OX1 3QU, UKNeuroscience, School of Medical Sciences, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Charles Perkins Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; John van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, CB2 0PY Cambridge, UKJohn van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, CB2 0PY Cambridge, UK; Blizard Institute, Barts and London School of Medicine and Dentistry, Queen Mary University London, UKSave Sight Institute, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Charles Perkins Centre, Faculty of Medicine and Health, University of Sydney, Sydney, AustraliaOxford Parkinson's Disease Centre and Department of Physiology, Anatomy & Genetics, Kavli Institute for Nanoscience Discovery, University of Oxford, Dorothy Crowfoot Hodgkin Building, South Parks Road, Oxford OX1 3QU, UKNeuroscience Drug Discovery, Ionis Pharmaceuticals, Inc., 2855 Gazelle Court, Carlsbad, CA 92010, USANuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UKDepartment of Clinical Sciences (DISCO), Section of Biochemistry, Polytechnic University of Marche, Via Ranieri 67, Ancona 60131, ItalyJohn van Geest Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge, Forvie Site, Robinson Way, CB2 0PY Cambridge, UK; Corresponding authors.Oxford Parkinson's Disease Centre and Department of Physiology, Anatomy & Genetics, Kavli Institute for Nanoscience Discovery, University of Oxford, Dorothy Crowfoot Hodgkin Building, South Parks Road, Oxford OX1 3QU, UK; Corresponding authors.SARM1 is a key regulator of a conserved program of axon degeneration increasingly linked to human neurodegenerative diseases. Pathological SARM1 activation causes rapid NAD consumption, disrupting cellular homeostasis and leading to axon degeneration. In this study, we develop antisense oligonucleotides (ASOs) targeting human SARM1, demonstrating robust neuroprotection against morphological, metabolic, and mitochondrial impairment in human iPSC-derived dopamine neurons induced by the lethal neurotoxin vacor, a potent SARM1 activator. Furthermore, our findings reveal that axon fragmentation can be prevented, and mitochondrial dysfunction reversed using the NAD precursor nicotinamide, a form of vitamin B3, even after SARM1 activation has occurred, when neurons are already unhealthy. This research identifies ASOs as a promising therapeutic strategy to block SARM1, and provides an extensive characterisation and further mechanistic insights that demonstrate the reversibility of SARM1 toxicity in human neurons. It also identifies the SARM1 activator vacor as a specific and reversible neuroablative agent in human neurons.http://www.sciencedirect.com/science/article/pii/S0969996125002025SARM1VacorASONicotinamideAxon degenerationMitochondrial dysfunction
spellingShingle Andrea Loreto
Kaitlyn M.L. Cramb
Lucy A. McDermott
Christina Antoniou
Ilenia Cirilli
Maria Claudia Caiazza
Elisa Merlini
Peter Arthur-Farraj
W. Daniel du Preez
Elliot D. Mock
Hien T. Zhao
David L. Bennett
Giuseppe Orsomando
Michael P. Coleman
Richard Wade-Martins
SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
Neurobiology of Disease
SARM1
Vacor
ASO
Nicotinamide
Axon degeneration
Mitochondrial dysfunction
title SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
title_full SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
title_fullStr SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
title_full_unstemmed SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
title_short SARM1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
title_sort sarm1 activation induces reversible mitochondrial dysfunction and can be prevented in human neurons by antisense oligonucleotides
topic SARM1
Vacor
ASO
Nicotinamide
Axon degeneration
Mitochondrial dysfunction
url http://www.sciencedirect.com/science/article/pii/S0969996125002025
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