Sandra Minotti
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View article: HOTAIR requires epitranscriptomic modification to exert its pivotal epigenetic role in Epithelial to Mesenchymal Transition
HOTAIR requires epitranscriptomic modification to exert its pivotal epigenetic role in Epithelial to Mesenchymal Transition Open
While m6A epitranscriptomic modification has been shown to impact several mRNAs maturation, stability/degradation, nuclear/cytoplasm export and translation regulation, its impact on lncRNAs activity is yet largely uncharacterized. Here, we…
View article: Iridium‐Bismuth‐Oxide Coatings for Use in Neural Stimulating Electrodes: The Influence of Ir/Bi Ratio
Iridium‐Bismuth‐Oxide Coatings for Use in Neural Stimulating Electrodes: The Influence of Ir/Bi Ratio Open
Implantable neural prosthetics with stimulating electrodes are increasingly employed in medical practices to treat neural disabilities. The electrode material is expected to provide high charge storage and injection capacity (CSC/CIC) and …
View article: Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis
Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis Open
Proteostasis is maintained through regulated protein synthesis and degradation and chaperone-assisted protein folding. However, this is challenging in neuronal projections because of their polarized morphology and constant synaptic proteom…
View article: CK1α, FAM83H, and FAM83B contribute to bundling of neurofilaments and are sequestered in cellular and mice models of ARSACS
CK1α, FAM83H, and FAM83B contribute to bundling of neurofilaments and are sequestered in cellular and mice models of ARSACS Open
Autosomal recessive spastic ataxia of the Charlevoix-Saguenay (ARSACS) is a rare neurodegenerative disorder characterized by mutations in the SACS gene that encodes for the sacsin protein. Sacsin dysfunction in ARSACS results in neurofilam…
View article: HDAC inhibitor SAHA resolved intermediate filament bundling and defective autophagy caused by the lack of sacsin in ARSACS.
HDAC inhibitor SAHA resolved intermediate filament bundling and defective autophagy caused by the lack of sacsin in ARSACS. Open
Autosomal recessive spastic ataxia of the Charlevoix-Saguenay (ARSACS) is a progressive childhood-onset neurological disease caused by loss-of-function mutations in the SACS gene. This condition is characterized by cerebellar ataxia, perip…
View article: Reversal of cognitive deficits in FUSR521G amyotrophic lateral sclerosis mice by arimoclomol and a class I histone deacetylase inhibitor independent of heat shock protein induction
Reversal of cognitive deficits in FUSR521G amyotrophic lateral sclerosis mice by arimoclomol and a class I histone deacetylase inhibitor independent of heat shock protein induction Open
Protein misfolding and mislocalization are common to both familial and sporadic forms of amyotrophic lateral sclerosis (ALS). Maintaining proteostasis through induction of heat shock proteins (HSP) to increase chaperoning capacity is a rat…
View article: Interactors of sacsin’s DNAJ domain identify function in organellar transport and membrane composition relevant to ARSACS pathogenesis
Interactors of sacsin’s DNAJ domain identify function in organellar transport and membrane composition relevant to ARSACS pathogenesis Open
Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS) is caused by loss of function mutations in the SACS gene encoding sacsin, a 520kDa protein with multiple functional domains. The goal of this study was to identify clie…
View article: Impact of histone deacetylase inhibition and arimoclomol on heat shock protein expression and disease biomarkers in primary culture models of familial ALS
Impact of histone deacetylase inhibition and arimoclomol on heat shock protein expression and disease biomarkers in primary culture models of familial ALS Open
Protein misfolding and mislocalization are common themes in neurodegenerative disorders, including the motor neuron disease, amyotrophic lateral sclerosis (ALS). Maintaining proteostasis is a crosscutting therapeutic target, including upre…
View article: Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis
Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis Open
Proteostasis is maintained through regulated protein synthesis and degradation and chaperone-assisted protein folding. However, this is challenging in neuronal projections because of their polarized morphology and constant synaptic proteom…
View article: The J Domain of Sacsin Disrupts Intermediate Filament Assembly
The J Domain of Sacsin Disrupts Intermediate Filament Assembly Open
Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS) is caused by mutation in the SACS gene resulting in loss of function of the protein sacsin. A key feature is the formation of abnormal bundles of neurofilaments (NF) in…
View article: The J domain of sacsin disrupts intermediate filament assembly
The J domain of sacsin disrupts intermediate filament assembly Open
Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS), is caused by loss of function mutations in the SACS gene, which encodes sacsin, a giant protein of 520 kDa. A key feature of the absence of sacsin in cells is the form…
View article: Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics
Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics Open
Loss of sacsin, a large 520 kDa multidomain protein, causes autosomal recessive spastic ataxia of the Charlevoix‐Saguenay, one of the most common childhood‐onset recessive ataxias. A prominent feature is abnormal bundling of neurofilaments…
View article: Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis
Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis Open
Amyotrophic lateral sclerosis is a fatal neurodegenerative disease with paralysis resulting from dysfunction and loss of motor neurons. A common neuropathological finding is attrition of motor neuron dendrites, which make central connectio…