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Neurofilaments: neurobiological foundations for biomarker applications

Brain (London, England : 1878), 2020-07, Vol.143 (7), p.1975-1998

2020

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TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

Scientific reports, 2018-05, Vol.8 (1), p.7551-13, Article 7551

2018

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Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo

Human molecular genetics, 2010-02, Vol.19 (4), p.671-683

2010

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Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics

The FASEB journal, 2019-02, Vol.33 (2), p.2982-2994

2019

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The J Domain of Sacsin Disrupts Intermediate Filament Assembly

International journal of molecular sciences, 2022-12, Vol.23 (24), p.15742

2022

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Progranulin is expressed within motor neurons and promotes neuronal cell survival

BMC neuroscience, 2009-10, Vol.10 (1), p.130-130, Article 130

2009

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Editorial: The Role of Heat Shock Proteins in Neuroprotection

Frontiers in pharmacology, 2020-08, Vol.11, p.1227-1227

2020

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Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

Neurobiology of disease, 2011-06, Vol.42 (3), p.265-275

2011

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Failure of protein quality control in amyotrophic lateral sclerosis

Biochimica et biophysica acta, 2006-11, Vol.1762 (11), p.1038-1050

2006

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The central role of heat shock factor 1 in synaptic fidelity and memory consolidation

Cell stress & chaperones, 2016-09, Vol.21 (5), p.745-753

2016

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Normal role of the low‐molecular‐weight neurofilament protein in mitochondrial dynamics and disruption in Charcot‐Marie‐Tooth disease

The FASEB journal, 2012-03, Vol.26 (3), p.1194-1203

2012

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Heterogeneity in the properties of NEFL mutants causing Charcot–Marie–Tooth disease results in differential effects on neurofilament assembly and susceptibility to intervention by the chaperone-inducer, celastrol

The international journal of biochemistry & cell biology, 2013-07, Vol.45 (7), p.1499-1508

2013

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In vitro study of axonal migration and myelination of motor neurons in a three-dimensional tissue-engineered model

Glia, 2008-02, Vol.56 (3), p.354-364

2008

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Neurofilament dynamics and involvement in neurological disorders

Cell and tissue research, 2015-06, Vol.360 (3), p.609-620

2015

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Functional Characterization of Neurofilament Light Splicing and Misbalance in Zebrafish

Cells (Basel, Switzerland), 2020-05, Vol.9 (5), p.1238

2020

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Induction of multiple heat shock proteins and neuroprotection in a primary culture model of familial amyotrophic lateral sclerosis

Neurobiology of disease, 2006-11, Vol.24 (2), p.213-225

2006

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Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis

Journal of neurochemistry, 2004-06, Vol.89 (6), p.1325-1335

2004

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Tryptophan 32 Potentiates Aggregation and Cytotoxicity of a Copper/Zinc Superoxide Dismutase Mutant Associated with Familial Amyotrophic Lateral Sclerosis

The Journal of biological chemistry, 2007-06, Vol.282 (22), p.16329-16335

2007

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Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations

Human molecular genetics, 2012-01, Vol.21 (1), p.136-149

2012

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A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivo

Cell stress & chaperones, 2014-05, Vol.19 (3), p.421-437

2014

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Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis

Human molecular genetics, 2017-11, Vol.26 (21), p.4142-4152

2017

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Glutamate Potentiates the Toxicity of Mutant Cu/Zn-Superoxide Dismutase in Motor Neurons by Postsynaptic Calcium-Dependent Mechanisms

The Journal of neuroscience, 1998-12, Vol.18 (23), p.9673-9684

1998

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Impact of histone deacetylase inhibition and arimoclomol on heat shock protein expression and disease biomarkers in primary culture models of familial ALS

Cell stress & chaperones, 2024-04, Vol.29 (3), p.359-380

2024

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Depending on the stress, histone deacetylase inhibitors act as heat shock protein co-inducers in motor neurons and potentiate arimoclomol, exerting neuroprotection through multiple mechanisms in ALS models

Cell stress & chaperones, 2020-01, Vol.25 (1), p.173-191

2020

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Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-Saguenay

Human molecular genetics, 2015-02, Vol.24 (3), p.727-739

2015

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Neurofilaments: neurobiological foundations for biomarker applications

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo

Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics

The J Domain of Sacsin Disrupts Intermediate Filament Assembly

Progranulin is expressed within motor neurons and promotes neuronal cell survival

Editorial: The Role of Heat Shock Proteins in Neuroprotection

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

Failure of protein quality control in amyotrophic lateral sclerosis

The central role of heat shock factor 1 in synaptic fidelity and memory consolidation

Normal role of the low‐molecular‐weight neurofilament protein in mitochondrial dynamics and disruption in Charcot‐Marie‐Tooth disease

Heterogeneity in the properties of NEFL mutants causing Charcot–Marie–Tooth disease results in differential effects on neurofilament assembly and susceptibility to intervention by the chaperone-inducer, celastrol

In vitro study of axonal migration and myelination of motor neurons in a three-dimensional tissue-engineered model

Neurofilament dynamics and involvement in neurological disorders

Functional Characterization of Neurofilament Light Splicing and Misbalance in Zebrafish

Induction of multiple heat shock proteins and neuroprotection in a primary culture model of familial amyotrophic lateral sclerosis

Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis

Tryptophan 32 Potentiates Aggregation and Cytotoxicity of a Copper/Zinc Superoxide Dismutase Mutant Associated with Familial Amyotrophic Lateral Sclerosis

Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations

A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivo

Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis

Glutamate Potentiates the Toxicity of Mutant Cu/Zn-Superoxide Dismutase in Motor Neurons by Postsynaptic Calcium-Dependent Mechanisms

Impact of histone deacetylase inhibition and arimoclomol on heat shock protein expression and disease biomarkers in primary culture models of familial ALS

Depending on the stress, histone deacetylase inhibitors act as heat shock protein co-inducers in motor neurons and potentiate arimoclomol, exerting neuroprotection through multiple mechanisms in ALS models

Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-Saguenay

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