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Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-SaguenayThe zinc- and calcium-binding S100B interacts and co-localizes with IQGAP1 during dynamic rearrangement of cell membranesA two-hybrid screen identifies an unconventional role for the intermediate filament peripherin in regulating the subcellular distribution of the SNAP25-interacting protein, SIP30The giant protein AHNAK is a specific target for the calcium- and zinc-binding S100B protein: potential implications for Ca2+ homeostasis regulation by S100B.A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivoMitochondrial morphology transitions and functions: implications for retrograde signaling?Expression of the protein chaperone, clusterin, in spinal cord cells constitutively and following cellular stress, and upregulation by treatment with Hsp90 inhibitorAcute exercise remodels mitochondrial membrane interactions in mouse skeletal muscle.Molecular basis of axonal dysfunction and traffic impairments in CMT.Neurofilament dynamics and involvement in neurological disorders.Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin.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.Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease.Specific AHNAK expression in brain endothelial cells with barrier properties.Commitment to apoptosis by ceramides depends on mitochondrial respiratory function, cytochrome c release and caspase-3 activation in Hep-G2 cells.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.Erratum to: 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.A New Mutation in FIG4 Causes a Severe Form of CMT4J Involving TRPV4 in the Pathogenic Cascade.Mechanical ventilation triggers abnormal mitochondrial dynamics and morphology in the diaphragm.The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.Expression of the Giant Protein AHNAK (Desmoyokin) in Muscle and Lining Epithelial Cells
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description
researcher ORCID id 0000-0003-2022-1890
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wetenschapper
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Benoit J Gentil
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Benoit J Gentil
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Benoit J Gentil
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Benoit J. Gentil
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Benoit J Gentil
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Benoit J Gentil
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Benoit J Gentil
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Benoit J. Gentil
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Benoit Gentil
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Benoit Gentil
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Benoit J Gentil
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Benoit J. Gentil
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Benoit J Gentil
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Benoit J Gentil
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Benoit J Gentil
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Benoit J. Gentil
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P106
P2038
Benoit_Gentil
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P496
0000-0003-2022-1890