List of works - Benoit J Gentil

A New Mutation in FIG4 Causes a Severe Form of CMT4J Involving TRPV4 in the Pathogenic Cascade

scientific article published in September 2017

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

scientific article

A two-hybrid screen identifies an unconventional role for the intermediate filament peripherin in regulating the subcellular distribution of the SNAP25-interacting protein, SIP30

scientific journal article

Acute exercise remodels mitochondrial membrane interactions in mouse skeletal muscle.

scientific article published on 22 August 2013

Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin

scientific article published on 23 May 2017

Commitment to apoptosis by ceramides depends on mitochondrial respiratory function, cytochrome c release and caspase-3 activation in Hep-G2 cells

scientific article published on December 2003

Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis

scientific article published on 26 July 2017

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

scientific article published on 19 November 2013

Expression of the Giant Protein AHNAK (Desmoyokin) in Muscle and Lining Epithelial Cells

scientific article published on 01 March 2003

Expression of the protein chaperone, clusterin, in spinal cord cells constitutively and following cellular stress, and upregulation by treatment with Hsp90 inhibitor

scientific article published on 19 April 2013

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

scientific article published on 22 April 2013

Mechanical ventilation triggers abnormal mitochondrial dynamics and morphology in the diaphragm

scientific article published on 12 March 2015

Mitochondrial and axonal abnormalities precede disruption of the neurofilament network in a model of charcot-marie-tooth disease type 2E and are prevented by heat shock proteins in a mutant-specific fashion.

scientific article

Mitochondrial morphology transitions and functions: implications for retrograde signaling?

scientific article published on 30 January 2013

Molecular basis of axonal dysfunction and traffic impairments in CMT.

scientific article published on 14 May 2012

Neurofilament dynamics and involvement in neurological disorders

scientific article published on 08 January 2015

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

scientific article

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

scientific article

Specific AHNAK expression in brain endothelial cells with barrier properties.

scientific article

The giant protein AHNAK is a specific target for the calcium- and zinc-binding S100B protein: potential implications for Ca2+ homeostasis regulation by S100B.

scientific article

The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.

scientific article published on 9 May 2014

The zinc- and calcium-binding S100B interacts and co-localizes with IQGAP1 during dynamic rearrangement of cell membranes

scientific article (publication date: 20 December 2002)

List of works by Benoit J Gentil

A New Mutation in FIG4 Causes a Severe Form of CMT4J Involving TRPV4 in the Pathogenic Cascade

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

A two-hybrid screen identifies an unconventional role for the intermediate filament peripherin in regulating the subcellular distribution of the SNAP25-interacting protein, SIP30

Acute exercise remodels mitochondrial membrane interactions in mouse skeletal muscle.

Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin

Commitment to apoptosis by ceramides depends on mitochondrial respiratory function, cytochrome c release and caspase-3 activation in Hep-G2 cells

Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis

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

Expression of the Giant Protein AHNAK (Desmoyokin) in Muscle and Lining Epithelial Cells

Expression of the protein chaperone, clusterin, in spinal cord cells constitutively and following cellular stress, and upregulation by treatment with Hsp90 inhibitor

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

Mechanical ventilation triggers abnormal mitochondrial dynamics and morphology in the diaphragm

Mitochondrial and axonal abnormalities precede disruption of the neurofilament network in a model of charcot-marie-tooth disease type 2E and are prevented by heat shock proteins in a mutant-specific fashion.

Mitochondrial morphology transitions and functions: implications for retrograde signaling?

Molecular basis of axonal dysfunction and traffic impairments in CMT.

Neurofilament dynamics and involvement in neurological disorders

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

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

Specific AHNAK expression in brain endothelial cells with barrier properties.

The giant protein AHNAK is a specific target for the calcium- and zinc-binding S100B protein: potential implications for Ca2+ homeostasis regulation by S100B.

The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.

The zinc- and calcium-binding S100B interacts and co-localizes with IQGAP1 during dynamic rearrangement of cell membranes