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Mass spectrometry imaging of SOD1 protein-metal complexes in SOD1G93A transgenic mice implicates demetalation with pathology

Nature communications, 2024-08, Vol.15 (1), p.6518-11, Article 6518

2024

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Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy

Science translational medicine, 2010-06, Vol.2 (35), p.35ra42

2010

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Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target

Biochimica et biophysica acta, 2006-11, Vol.1762 (11), p.1051-1067

2006

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Advances, challenges and future directions for stem cell therapy in amyotrophic lateral sclerosis

Molecular neurodegeneration, 2017-11, Vol.12 (1), p.85-85, Article 85

2017

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A Y374X TDP43 truncation leads to an altered metabolic profile in amyotrophic lateral sclerosis fibroblasts driven by pyruvate and TCA cycle intermediate alterations

Frontiers in aging neuroscience, 2023-05, Vol.15, p.1151848

2023

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Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

Nature reviews. Drug discovery, 2023-03, Vol.22 (3), p.185-212

2023

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Extensive phenotypic characterisation of a human TDP-43Q331K transgenic mouse model of amyotrophic lateral sclerosis (ALS)

Scientific reports, 2021-08, Vol.11 (1), p.16659-16659, Article 16659

2021

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The GLP-1 receptor agonist, liraglutide, fails to slow disease progression in SOD1G93A and TDP-43Q331K transgenic mouse models of ALS

Scientific reports, 2021-08, Vol.11 (1), p.17027-17027, Article 17027

2021

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Author Correction: Female sex mitigates motor and behavioural phenotypes in TDP-43Q331K knock-in mice

Scientific reports, 2021-05, Vol.11 (1), p.11058-11058, Article 11058

2021

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Adipose-derived stem cells protect motor neurons and reduce glial activation in both in vitro and in vivo models of ALS

Molecular therapy. Methods & clinical development, 2021-06, Vol.21, p.413-433

2021

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Janus kinase inhibitors are potential therapeutics for amyotrophic lateral sclerosis

Translational neurodegeneration, 2023-10, Vol.12 (1), p.47-47, Article 47

2023

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Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS)

PloS one, 2011, Vol.6 (8), p.e23244

2011

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Caudal–Rostral Progression of Alpha Motoneuron Degeneration in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

Antioxidants, 2022-05, Vol.11 (5), p.983

2022

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Confocal Endomicroscopy of Neuromuscular Junctions Stained with Physiologically Inert Protein Fragments of Tetanus Toxin

Biomolecules (Basel, Switzerland), 2021-10, Vol.11 (10), p.1499

2021

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Early detection of motor dysfunction in the SOD1G93A mouse model of Amyotrophic Lateral Sclerosis (ALS) using home cage running wheels

PloS one, 2014, Vol.9 (9), p.e107918-e107918

2014

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Combining electromyography and Raman spectroscopy: optical EMG

Muscle & nerve, 2023-10, Vol.68 (4), p.464-470

2023

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Conformational fingerprinting with Raman spectroscopy reveals protein structure as a translational biomarker of muscle pathology

Analyst (London), 2024-04, Vol.149 (9), p.2738-2746

2024

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Fiber optic Raman spectroscopy for the evaluation of disease state in Duchenne muscular dystrophy: An assessment using the mdx model and human muscle

Muscle & nerve, 2022-09, Vol.66 (3), p.362-369

2022

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Assessment of the Precision in Measuring Glutathione at 3 T With a MEGA‐PRESS Sequence in Primary Motor Cortex and Occipital Cortex

Journal of magnetic resonance imaging, 2022-02, Vol.55 (2), p.435-442

2022

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A cell-penetrant peptide blocking C9ORF72 -repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins

Science translational medicine, 2023-03, Vol.15 (685), p.eabo3823

2023

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Atypical TDP‐43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP

Brain pathology (Zurich, Switzerland), 2023-01, Vol.33 (1), p.e13104-n/a

2023

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Female sex mitigates motor and behavioural phenotypes in TDP-43Q331K knock-in mice

Scientific reports, 2020-11, Vol.10 (1), p.19220-19220, Article 19220

2020

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Non‐negative matrix factorisation of Raman spectra finds common patterns relating to neuromuscular disease across differing equipment configurations, preclinical models and human tissue

Journal of Raman spectroscopy, 2023-03, Vol.54 (3), p.258-268

2023

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S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis

Free radical biology & medicine, 2013-08, Vol.61, p.438-452

2013

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An in vitro screening cascade to identify neuroprotective antioxidants in ALS

Free radical biology & medicine, 2009-04, Vol.46 (8), p.1127-1138

2009

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Mass spectrometry imaging of SOD1 protein-metal complexes in SOD1G93A transgenic mice implicates demetalation with pathology

Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy

Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target

Advances, challenges and future directions for stem cell therapy in amyotrophic lateral sclerosis

A Y374X TDP43 truncation leads to an altered metabolic profile in amyotrophic lateral sclerosis fibroblasts driven by pyruvate and TCA cycle intermediate alterations

Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

Extensive phenotypic characterisation of a human TDP-43Q331K transgenic mouse model of amyotrophic lateral sclerosis (ALS)

The GLP-1 receptor agonist, liraglutide, fails to slow disease progression in SOD1G93A and TDP-43Q331K transgenic mouse models of ALS

Author Correction: Female sex mitigates motor and behavioural phenotypes in TDP-43Q331K knock-in mice

Adipose-derived stem cells protect motor neurons and reduce glial activation in both in vitro and in vivo models of ALS

Janus kinase inhibitors are potential therapeutics for amyotrophic lateral sclerosis

Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS)

Caudal–Rostral Progression of Alpha Motoneuron Degeneration in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

Confocal Endomicroscopy of Neuromuscular Junctions Stained with Physiologically Inert Protein Fragments of Tetanus Toxin

Early detection of motor dysfunction in the SOD1G93A mouse model of Amyotrophic Lateral Sclerosis (ALS) using home cage running wheels

Combining electromyography and Raman spectroscopy: optical EMG

Conformational fingerprinting with Raman spectroscopy reveals protein structure as a translational biomarker of muscle pathology

Fiber optic Raman spectroscopy for the evaluation of disease state in Duchenne muscular dystrophy: An assessment using the mdx model and human muscle

Assessment of the Precision in Measuring Glutathione at 3 T With a MEGA‐PRESS Sequence in Primary Motor Cortex and Occipital Cortex

A cell-penetrant peptide blocking C9ORF72 -repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins

Atypical TDP‐43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP

Female sex mitigates motor and behavioural phenotypes in TDP-43Q331K knock-in mice

Non‐negative matrix factorisation of Raman spectra finds common patterns relating to neuromuscular disease across differing equipment configurations, preclinical models and human tissue

S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis

An in vitro screening cascade to identify neuroprotective antioxidants in ALS

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