Bernard L. Schneider
YOU?
Author Swipe
View article: Mifepristone alone and in combination with scAAV9-SMN1 gene therapy improves disease phenotypes in Smn2B/- spinal muscular atrophy mice
Mifepristone alone and in combination with scAAV9-SMN1 gene therapy improves disease phenotypes in Smn2B/- spinal muscular atrophy mice Open
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by deletions or mutations in the survival motor neuron 1 ( SMN1 ) gene. SMA is characterised by alpha motor neuron loss in the spinal cord and subsequent muscle atrophy. There…
View article: Gene therapy-mediated overexpression of wild-type MFN2 improves Charcot-Marie-Tooth disease type 2A
Gene therapy-mediated overexpression of wild-type MFN2 improves Charcot-Marie-Tooth disease type 2A Open
Charcot-Marie-Tooth disease type 2A (CMT2A) is the most common axonal CMT and is associated with an early onset and severe motor-dominant phenotype. CMT2A is mainly caused by dominant mutations in the MFN2 gene, encoding Mitofusin-2, a GTP…
View article: NATO3 protects dopaminergic neurons in mouse <i>in vivo</i> and human <i>in vitro</i> Parkinson’s disease models
NATO3 protects dopaminergic neurons in mouse <i>in vivo</i> and human <i>in vitro</i> Parkinson’s disease models Open
Parkinson’s disease (PD) is a devastating neurodegenerative disorder primarily characterized by the progressive and unstoppable loss of dopaminergic (DA) neurons in the substantia nigra. We previously identified NATO3 (FERD3L), a conserved…
View article: One-shot design of functional protein binders with BindCraft
One-shot design of functional protein binders with BindCraft Open
View article: Highly frequent undesired insertional mutagenesis during Drosophila genome editing
Highly frequent undesired insertional mutagenesis during Drosophila genome editing Open
CRISPR/Cas9 based genome editing employing Homology Directed Repair (HDR) from template vector sequences is a widely used technique to enable precise insertions, deletions or modifications to genes. Here, we describe an undesired and highl…
View article: Optogenetic modulation of peripheral nociceptive neurons with biocompatible optoelectronic implants
Optogenetic modulation of peripheral nociceptive neurons with biocompatible optoelectronic implants Open
Hyperexcitability of peripheral sensory neurons plays a critical role in the development and maintenance of chronic pain. Pharmacological analgesics used in clinics reduce neuronal activity. They often come with non‐negligible side effects…
View article: Cerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration
Cerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration Open
View article: Mifepristone alone and in combination with scAAV9-<i>SMN1</i> gene therapy improves disease phenotypes in <i>Smn<sup>2B/-</sup></i>spinal muscular atrophy mice
Mifepristone alone and in combination with scAAV9-<i>SMN1</i> gene therapy improves disease phenotypes in <i>Smn<sup>2B/-</sup></i>spinal muscular atrophy mice Open
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by deletions or mutations in the survival motor neuron 1 ( SMN1 ) gene. SMA is characterised by alpha motor neuron loss in the spinal cord and subsequent muscle atrophy. There…
View article: Interactions of VMAT2 with CDCrel-1 and Parkin in Methamphetamine Neurotoxicity
Interactions of VMAT2 with CDCrel-1 and Parkin in Methamphetamine Neurotoxicity Open
In recent years, methamphetamine (METH) misuse in the US has been rapidly increasing, and there is no FDA-approved pharmacotherapy for METH use disorder (MUD). In addition to being dependent on the drug, people with MUD develop a variety o…
View article: Liver SMN restoration rescues the Smn mouse model of spinal muscular atrophy
Liver SMN restoration rescues the Smn mouse model of spinal muscular atrophy Open
This work was funded by Muscular Dystrophy Association (USA) [grant number 963652 to R.K.]; the Canadian Institutes of Health Research [grant number PJT-186300 to R.K.].
View article: TNFα prevents FGF4-mediated rescue of astrocyte dysfunction and reactivity in human ALS models
TNFα prevents FGF4-mediated rescue of astrocyte dysfunction and reactivity in human ALS models Open
View article: BindCraft: one-shot design of functional protein binders
BindCraft: one-shot design of functional protein binders Open
Protein–protein interactions (PPIs) are at the core of all key biological processes. However, the complexity of the structural features that determine PPIs makes their design challenging. We present BindCraft, an open-source and automated …
View article: Single-cell and spatial atlases of spinal cord injury in the Tabulae Paralytica
Single-cell and spatial atlases of spinal cord injury in the Tabulae Paralytica Open
View article: Production and Purification of Adeno-Associated Viral Vectors (AAVs) Using Orbitally Shaken HEK293 Cells
Production and Purification of Adeno-Associated Viral Vectors (AAVs) Using Orbitally Shaken HEK293 Cells Open
View article: Liver Smn Restoration Rescues the Smn2b/- Mouse Model of Spinal Muscular Atrophy: The Key to Rescue Whole Body Pathology?
Liver Smn Restoration Rescues the Smn2b/- Mouse Model of Spinal Muscular Atrophy: The Key to Rescue Whole Body Pathology? Open
View article: Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy
Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy Open
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by motor neuron loss and skeletal muscle atrophy. SMA is caused by the loss of the SMN1 gene and low SMN protein levels. Current SMA therapies work…
View article: TNFα hinders FGF4 efficacy to mitigate ALS astrocyte dysfunction and cGAS-STING pathway-induced innate immune reactivity
TNFα hinders FGF4 efficacy to mitigate ALS astrocyte dysfunction and cGAS-STING pathway-induced innate immune reactivity Open
Astrocytes play an important role in the onset and progression of amyotrophic lateral sclerosis (ALS), a fatal disorder characterized by the relentless degeneration of motor neurons (MNs) in the central nervous system. Despite evidence sho…
View article: Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth
Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth Open
Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are the most common motoneuron diseases affecting adults and infants, respectively. ALS and SMA are both characterized by the selective degeneration of motoneurons. Alth…
View article: Stable isotope labeling and ultra-high-resolution NanoSIMS imaging reveal alpha-synuclein-induced changes in neuronal metabolism in vivo
Stable isotope labeling and ultra-high-resolution NanoSIMS imaging reveal alpha-synuclein-induced changes in neuronal metabolism in vivo Open
View article: Recovery of walking after paralysis by regenerating characterized neurons to their natural target region
Recovery of walking after paralysis by regenerating characterized neurons to their natural target region Open
Axon regeneration can be induced across anatomically complete spinal cord injury (SCI), but robust functional restoration has been elusive. Whether restoring neurological functions requires directed regeneration of axons from specific neur…
View article: AAV9-mediated <i>SMN</i> gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in <i>Smn</i> <i>2B/−</i> spinal muscular atrophy mice
AAV9-mediated <i>SMN</i> gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in <i>Smn</i> <i>2B/−</i> spinal muscular atrophy mice Open
Structural, functional and molecular cardiac defects have been reported in spinal muscular atrophy (SMA) patients and mouse models. Previous quantitative proteomics analyses demonstrated widespread molecular defects in the severe Taiwanese…
View article: Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function
Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function Open
The nucleus accumbens (NAc) is a brain hub regulating motivated behaviors, including social competitiveness. Mitochondrial function in the NAc links anxiety with social competitiveness, and the mitochondrial fusion protein mitofusin 2 (Mfn…
View article: <i>The Tabulae Paralytica:</i>Multimodal single-cell and spatial atlases of spinal cord injury
<i>The Tabulae Paralytica:</i>Multimodal single-cell and spatial atlases of spinal cord injury Open
Here, we introduce the Tabulae Paralytica —a compilation of four atlases of spinal cord injury (SCI) comprising a single-nucleus transcriptome atlas of half a million cells; a multiome atlas pairing transcriptomic and epigenomic measuremen…
View article: Rapid ex vivo reverse genetics identifies the essential determinants of prion protein toxicity
Rapid ex vivo reverse genetics identifies the essential determinants of prion protein toxicity Open
The cellular prion protein PrP C mediates the neurotoxicity of prions and other protein aggregates through poorly understood mechanisms. Antibody‐derived ligands against the globular domain of PrP C (GDL) can also initiate neurotoxicity by…
View article: Rapid ex vivo reverse genetics identifies the essential determinants of prion protein toxicity
Rapid ex vivo reverse genetics identifies the essential determinants of prion protein toxicity Open
The cellular prion protein PrP C mediates the neurotoxicity of prions and other protein aggregates through poorly understood mechanisms. Antibody-derived ligands against the globular domain of PrP C (GDL) can also initiate neurotoxicity by…
View article: Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function
Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function Open
Summary The nucleus accumbens (NAc) is a brain hub regulating motivated behaviors, including social competitiveness. Mitochondrial function in the NAc is critically implicated in the association between anxiety and social competitiveness, …
View article: Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth
Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth Open
Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are the most common motoneuron diseases affecting adults and infants, respectively. ALS and SMA are both characterized by the selective degeneration of motoneurons. Alth…
View article: Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy
Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy Open
View article: Astrocyte‐targeting <scp>RNA</scp> interference against mutated superoxide dismutase 1 induces motoneuron plasticity and protects fast‐fatigable motor units in a mouse model of amyotrophic lateral sclerosis
Astrocyte‐targeting <span>RNA</span> interference against mutated superoxide dismutase 1 induces motoneuron plasticity and protects fast‐fatigable motor units in a mouse model of amyotrophic lateral sclerosis Open
In amyotrophic lateral sclerosis (ALS) caused by SOD1 gene mutations, both cell‐autonomous and noncell‐autonomous mechanisms lead to the selective degeneration of motoneurons (MN). Here, we evaluate the therapeutic potential of gene therap…
View article: Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function
Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function Open
The nucleus accumbens (NAc) is a brain hub regulating motivated behaviors, including social competitiveness. Mitochondrial function in the NAc is critically implicated in the association between anxiety and social competitiveness, and the …