List of works - Jean-Pierre Julien

A NUDEL-dependent mechanism of neurofilament assembly regulates the integrity of CNS neurons

scientific journal article

A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis

scientific article

A neurotoxic peripherin splice variant in a mouse model of ALS

scientific journal article

ALS pathogenesis: recent insights from genetics and mouse models

scientific article published on 20 August 2010

ALS: astrocytes move in as deadly neighbors

scientific article

Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase

scientific article

Ablation of proliferating microglia does not affect motor neuron degeneration in amyotrophic lateral sclerosis caused by mutant superoxide dismutase.

scientific article

Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis

scientific article published on 01 December 2012

Absence of tumor necrosis factor-alpha does not affect motor neuron disease caused by superoxide dismutase 1 mutations

scientific article published on 01 November 2006

Adeno-associated virus-mediated delivery of a recombinant single-chain antibody against misfolded superoxide dismutase for treatment of amyotrophic lateral sclerosis

scientific article published on 17 October 2013

Aggregate formation and phosphorylation of neurofilament-L Pro22 Charcot-Marie-Tooth disease mutants

scientific article published on February 2006

Alpha-internexin is structurally and functionally associated with the neurofilament triplet proteins in the mature CNS.

scientific article published in September 2006

Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish

scientific article published on 16 June 2008

Alsin is partially associated with centrosome in human cells

scientific article

Altered immune response to CNS viral infection in mice with a conditional knock-down of macrophage-lineage cells

scientific article

Axonal transport deficits and neurodegenerative diseases ⬇️

scientific article published on January 30, 2013

Bone marrow-derived microglia play a critical role in restricting senile plaque formation in Alzheimer's disease

scientific article published in February 2006

CD11b+Ly6G- myeloid cells mediate mechanical inflammatory pain hypersensitivity.

scientific article

Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1.

scientific article published in March 2003

Ceruloplasmin regulates iron levels in the CNS and prevents free radical injury.

scientific article published in August 2002

Chapter 6 Animal models of motor neuron death

scientific article published on 01 January 2007

Chromogranin B P413L variant as risk factor and modifier of disease onset for amyotrophic lateral sclerosis

scientific article

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

scientific article

Chronic Administration of Pimozide Fails to Attenuate Motor and Pathological Deficits in Two Mouse Models of Amyotrophic Lateral Sclerosis

scientific article published on 01 July 2018

Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects

Conditional NF-L transgene expression in mice for in vivo analysis of turnover and transport rate of neurofilaments

scientific article

Control of dendritic cell cross-presentation by the major histocompatibility complex class I cytoplasmic domain ⬇️

scientific article published on October 19, 2003

Cyclin-dependent kinase 5 in amyotrophic lateral sclerosis

scientific article published on September 2003

Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease).

scientific article

Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin

scientific article published on 19 June 2006

Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways

scientific article

Disruption of neurofilament network with aggregation of light neurofilament protein: a common pathway leading to motor neuron degeneration due to Charcot-Marie-Tooth disease-linked mutations in NFL and HSPB1.

scientific article published on 19 September 2007

Dissociation of Axonal Neurofilament Content from Its Transport Rate

scientific article published on 24 July 2015

Early and persistent abnormal decoding by glial cells at the neuromuscular junction in an ALS model

scientific article published on 01 January 2015

Early-stage treatment with Withaferin A reduces levels of misfolded superoxide dismutase 1 and extends lifespan in a mouse model of amyotrophic lateral sclerosis

scientific article

Efficient three-drug cocktail for disease induced by mutant superoxide dismutase

scientific article published in April 2003

Exacerbation of motor neuron disease by chronic stimulation of innate immunity in a mouse model of amyotrophic lateral sclerosis.

scientific article published in February 2004

Exosome secretion is a key pathway for clearance of pathological TDP-43.

scientific article published on 27 September 2016

Expression of Human Neurofilament-light Transgene in Mouse Neurons Transplanted into the Brain of Adult Rats

scientific article published on 01 January 1991

Extracellular mutant SOD1 induces microglial-mediated motoneuron injury

scientific article published on January 2010

From animal models to human disease: a genetic approach for personalized medicine in ALS

scientific article

Functions of intermediate filaments in neuronal development and disease ⬇️

scientific article published on January 1, 2004

Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation

scientific article published on 15 April 2013

Gigaxonin mutation analysis in patients with NIFID.

scientific article published on 26 September 2009

Increased glioma growth in mice depleted of macrophages.

scientific article

Induction of autophagy mitigates TDP-43 pathology and translational repression of neurofilament mRNAs in mouse models of ALS/FTD

scientific article published on 07 January 2021

Induction of peripherin expression in subsets of brain neurons after lesion injury or cerebral ischemia

scientific article published in August 2002

Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice

scientific article published on 01 October 2010

Inflammation Induces TDP-43 Mislocalization and Aggregation

scientific article published on 7 October 2015

Innate immunity: the missing link in neuroprotection and neurodegeneration?

scientific article

Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits

scientific article

Intermediate filament aggregates cause mitochondrial dysmotility and increase energy demands in giant axonal neuropathy

scientific article

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

scientific article published on 14 March 2010

Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia

scientific article published on 18 July 2019

Local Acceleration of Neurofilament Transport at Nodes of Ranvier

scientific article published on 12 December 2018

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS ⬇️

scientific article published on March 19, 2012

Loss of glial neurofascin155 delays developmental synapse elimination at the neuromuscular junction

scientific article

MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis

scientific article published on 2 November 2015

Macrophage colony stimulating factor (M-CSF) exacerbates ALS disease in a mouse model through altered responses of microglia expressing mutant superoxide dismutase.

scientific article

Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis

scientific article published in August 2002

Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset.

scientific article

Misfolded SOD1 pathology in sporadic Amyotrophic Lateral Sclerosis

scientific article published in Scientific Reports

Mitigation of ALS pathology by neuron-specific inhibition of nuclear factor kappa B signaling

scientific article published on 26 May 2020

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

scientific article published on 04 June 2013

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

scientific article published on 4 October 2013

Modest loss of peripheral axons, muscle atrophy and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1

scientific article published on 31 July 2008

Monoclonal full-length antibody against TAR DNA-binding protein 43 reduces related proteinopathy in neurons

scientific article published on 06 October 2020

Mouse model for ablation of proliferating microglia in acute CNS injuries.

scientific article published in February 2006

Myosin Va binding to neurofilaments is essential for correct myosin Va distribution and transport and neurofilament density

scientific journal article

Natural history of node-negative breast cancer: are conventional prognostic factors predictors of time to relapse?

scientific article published on 01 October 2002

Neurofilament depletion improves microtubule dynamics via modulation of Stat3/stathmin signaling

scientific article published on 28 March 2016

Neurofilament light interaction with GluN1 modulates neurotransmission and schizophrenia-associated behaviors

Neurofilament transport in vivo minimally requires hetero-oligomer formation

scientific journal article

Neuroleptics as therapeutic compounds stabilizing neuromuscular transmission in amyotrophic lateral sclerosis.

scientific article

Neuronal Expression of UBQLN2 Exacerbates TDP-43 Pathology in TDP-43 Mice through Interaction with Ubiquitin

scientific article published on 30 October 2018

Neuronal MEK is important for normal fear conditioning in mice.

scientific article published on March 2004

Neuronal expression of peripherin, a type III intermediate filament protein, in the mouse hindbrain

scientific article published on 26 September 2007

Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS

scientific article published on 26 October 2010

Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival

scientific article published on 2 October 2013

New movements in neurofilament transport, turnover and disease.

scientific article published on 21 March 2007

Normal dendritic arborization in spinal motoneurons requires neurofilament subunit L

scientific article published on 01 August 2002

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

scientific article

Origin of the two mRNA species for the human neurofilament light gene

scientific article published on May 1, 1992

Overexpressed wild-type superoxide dismutase 1 exhibits amyotrophic lateral sclerosis-related misfolded conformation in induced pluripotent stem cell-derived spinal motor neurons

scientific article published on 14 November 2017

Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments ⬇️

scientific article published on July 13, 2011

Pathways to motor neuron degeneration in transgenic mouse models

scientific article

Peripherin Is a Subunit of Peripheral Nerve Neurofilaments: Implications for Differential Vulnerability of CNS and Peripheral Nervous System Axons ⬇️

scientific article published on June 20, 2012

Peripherin is not a contributing factor to motor neuron disease in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase.

scientific article

Peripherin-mediated death of motor neurons rescued by overexpression of neurofilament NF-H proteins

scientific article published on 01 April 2003

Preoperative chemotherapy is safe in early breast cancer, even after 10 years of follow-up; clinical and translational results from the EORTC trial 10902.

scientific article published on 18 May 2008

Protective effects of Withania somnifera extract in SOD1G93A mouse model of amyotrophic lateral sclerosis

scientific article published on 19 August 2018

Real-time imaging reveals defects of fast axonal transport induced by disorganization of intermediate filaments

scientific article published on 18 May 2009

Reply to van Vught et al.: P413L CHGB as risk factor for ALS.

scientific article published on 29 April 2010

Requirement of myeloid cells for axon regeneration

scientific article

Reversal of neuropathy phenotypes in conditional mouse model of Charcot-Marie-Tooth disease type 2E.

scientific article published on 26 April 2010

STochastic Optical Reconstruction Microscopy (STORM) reveals the nanoscale organization of pathological aggregates in human brain

scientific article published on 20 July 2020

Sensorimotor and cognitive function of a NEFLP22S mutant model of Charcot–Marie–Tooth disease type 2E ⬇️

scientific article published on December 17, 2010

Sex-dependent effects of chromogranin B P413L allelic variant as disease modifier in amyotrophic lateral sclerosis

scientific article

The Src/c-Abl pathway is a potential therapeutic target in amyotrophic lateral sclerosis.

scientific article

The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS.

scientific article

The high and middle molecular weight neurofilament subunits regulate the association of neurofilaments with kinesin: inhibition by phosphorylation of the high molecular weight subunit

scientific article

The myosin Va head domain binds to the neurofilament-L rod and modulates endoplasmic reticulum (ER) content and distribution within axons

scientific article

The single neurofilament subunit of lamprey may need another element for filament assembly

scientific article published on March 2004

The type III neurofilament peripherin is expressed in the tuberomammillary neurons of the mouse

scientific article

Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis

scholarly article

Therapeutic immunization with a glatiramer acetate derivative does not alter survival in G93A and G37R SOD1 mouse models of familial ALS

scientific article published on 30 December 2006

Transgenic mouse models of amyotrophic lateral sclerosis

scientific article

Transmission of ALS pathogenesis by the cerebrospinal fluid

scientific article published on 07 May 2020

Type II spiral ganglion afferent neurons drive medial olivocochlear reflex suppression of the cochlear amplifier

scientific article

Type III intermediate filament peripherin inhibits neuritogenesis in type II spiral ganglion neurons in vitro

scientific article published on 2 February 2010

Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.

scientific article

Up-regulation of peripherin is associated with alterations in synaptic plasticity in CA1 and CA3 regions of hippocampus

scientific article

Ureteric Bud Apoptosis and Renal Hypoplasia in Transgenic PAX2-Bax Fetal Mice Mimics the Renal-Coloboma Syndrome ⬇️

scientific article published on November 1, 2003

Virus-mediated delivery of antibody targeting TAR DNA-binding protein-43 mitigates associated neuropathology

scientific article published on 25 February 2019

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS ⬇️

scientific article published on October 17, 2010

Wild-type human SOD1 overexpression does not accelerate motor neuron disease in mice expressing murine Sod1 G86R.

scientific article published on 2 June 2010

Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation

scientific article published on 29 March 2007

Withaferin-A Treatment Alleviates TAR DNA-Binding Protein-43 Pathology and Improves Cognitive Function in a Mouse Model of FTLD

scientific article published on 19 October 2020

Withania somnifera Reverses Transactive Response DNA Binding Protein 43 Proteinopathy in a Mouse Model of Amyotrophic Lateral Sclerosis/Frontotemporal Lobar Degeneration.

scientific article

[35S]Methionine metabolic labeling to study axonal transport of neuronal intermediate filament proteins in vivo.

scientific article published in January 2004

[Dysfunction of axonal transport in neuropathies and motor neuron diseases]

scientific article published on 01 January 2008

Étude du cerveau humain à l’échelle nanoscopique par microscopie Super-Résolutive STORM

scientific article published on 01 September 2020

List of works by Jean-Pierre Julien

A NUDEL-dependent mechanism of neurofilament assembly regulates the integrity of CNS neurons

A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis

A neurotoxic peripherin splice variant in a mouse model of ALS

ALS pathogenesis: recent insights from genetics and mouse models

ALS: astrocytes move in as deadly neighbors

Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase

Ablation of proliferating microglia does not affect motor neuron degeneration in amyotrophic lateral sclerosis caused by mutant superoxide dismutase.

Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis

Absence of tumor necrosis factor-alpha does not affect motor neuron disease caused by superoxide dismutase 1 mutations

Adeno-associated virus-mediated delivery of a recombinant single-chain antibody against misfolded superoxide dismutase for treatment of amyotrophic lateral sclerosis

Aggregate formation and phosphorylation of neurofilament-L Pro22 Charcot-Marie-Tooth disease mutants

Alpha-internexin is structurally and functionally associated with the neurofilament triplet proteins in the mature CNS.

Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish

Alsin is partially associated with centrosome in human cells

Altered immune response to CNS viral infection in mice with a conditional knock-down of macrophage-lineage cells

Axonal transport deficits and neurodegenerative diseases ⬇️

Bone marrow-derived microglia play a critical role in restricting senile plaque formation in Alzheimer's disease

CD11b+Ly6G- myeloid cells mediate mechanical inflammatory pain hypersensitivity.

Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1.

Ceruloplasmin regulates iron levels in the CNS and prevents free radical injury.

Chapter 6 Animal models of motor neuron death

Chromogranin B P413L variant as risk factor and modifier of disease onset for amyotrophic lateral sclerosis

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

Chronic Administration of Pimozide Fails to Attenuate Motor and Pathological Deficits in Two Mouse Models of Amyotrophic Lateral Sclerosis

Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects

Conditional NF-L transgene expression in mice for in vivo analysis of turnover and transport rate of neurofilaments

Control of dendritic cell cross-presentation by the major histocompatibility complex class I cytoplasmic domain ⬇️

Cyclin-dependent kinase 5 in amyotrophic lateral sclerosis

Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease).

Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin

Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways

Disruption of neurofilament network with aggregation of light neurofilament protein: a common pathway leading to motor neuron degeneration due to Charcot-Marie-Tooth disease-linked mutations in NFL and HSPB1.

Dissociation of Axonal Neurofilament Content from Its Transport Rate

Early and persistent abnormal decoding by glial cells at the neuromuscular junction in an ALS model

Early-stage treatment with Withaferin A reduces levels of misfolded superoxide dismutase 1 and extends lifespan in a mouse model of amyotrophic lateral sclerosis

Efficient three-drug cocktail for disease induced by mutant superoxide dismutase

Exacerbation of motor neuron disease by chronic stimulation of innate immunity in a mouse model of amyotrophic lateral sclerosis.

Exosome secretion is a key pathway for clearance of pathological TDP-43.

Expression of Human Neurofilament-light Transgene in Mouse Neurons Transplanted into the Brain of Adult Rats

Extracellular mutant SOD1 induces microglial-mediated motoneuron injury

From animal models to human disease: a genetic approach for personalized medicine in ALS

Functions of intermediate filaments in neuronal development and disease ⬇️

Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation

Gigaxonin mutation analysis in patients with NIFID.

Increased glioma growth in mice depleted of macrophages.

Induction of autophagy mitigates TDP-43 pathology and translational repression of neurofilament mRNAs in mouse models of ALS/FTD

Induction of peripherin expression in subsets of brain neurons after lesion injury or cerebral ischemia

Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice

Inflammation Induces TDP-43 Mislocalization and Aggregation

Innate immunity: the missing link in neuroprotection and neurodegeneration?

Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits

Intermediate filament aggregates cause mitochondrial dysmotility and increase energy demands in giant axonal neuropathy

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia

Local Acceleration of Neurofilament Transport at Nodes of Ranvier

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS ⬇️

Loss of glial neurofascin155 delays developmental synapse elimination at the neuromuscular junction

MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis

Macrophage colony stimulating factor (M-CSF) exacerbates ALS disease in a mouse model through altered responses of microglia expressing mutant superoxide dismutase.

Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis

Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset.

Misfolded SOD1 pathology in sporadic Amyotrophic Lateral Sclerosis

Mitigation of ALS pathology by neuron-specific inhibition of nuclear factor kappa B signaling

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

Modest loss of peripheral axons, muscle atrophy and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1

Monoclonal full-length antibody against TAR DNA-binding protein 43 reduces related proteinopathy in neurons

Mouse model for ablation of proliferating microglia in acute CNS injuries.

Myosin Va binding to neurofilaments is essential for correct myosin Va distribution and transport and neurofilament density

Natural history of node-negative breast cancer: are conventional prognostic factors predictors of time to relapse?

Neurofilament depletion improves microtubule dynamics via modulation of Stat3/stathmin signaling

Neurofilament light interaction with GluN1 modulates neurotransmission and schizophrenia-associated behaviors

Neurofilament transport in vivo minimally requires hetero-oligomer formation

Neuroleptics as therapeutic compounds stabilizing neuromuscular transmission in amyotrophic lateral sclerosis.

Neuronal Expression of UBQLN2 Exacerbates TDP-43 Pathology in TDP-43 Mice through Interaction with Ubiquitin

Neuronal MEK is important for normal fear conditioning in mice.

Neuronal expression of peripherin, a type III intermediate filament protein, in the mouse hindbrain

Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS

Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival