Publications by Bernard Schneider

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease wherein motor neu-ron degeneration leads to muscle weakness, progressive paralysis, and death within 3–5 years ofdiagnosis. Currently, the cause of ALS is unknown but, as with several neuro ...

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. Although different in ...

Methamphetamine (METH) is a highly abused psychostimulant that is neurotoxic to dopaminergic (DAergic) nerve terminals in the striatum and increases the risk of developing Parkinson's disease (PD). In vivo, METH-mediated DA release, followed by DA-mediated ...

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 by increasing SM ...

Viral delivery of exogenous coding sequences into the inner ear has the potential for therapeutic benefit for patients suffering genetic or acquired hearing loss. To devise improved strategies for viral delivery, we investigated two injection techniques, r ...

We developed an ex vivo gene therapy approach for the regulated delivery of therapeutic proteins based on the implantation of encapsulated, genetically engineered C(2)C(12) myoblasts. We investigated doxycycline-based regulation of gene expression to modul ...

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 measurements within the same ...

There is no FDA-approved medication for methamphetamine (METH) use disorder. New therapeutic approaches are needed, especially for people who use METH heavily and are at high risk for overdose. This study used genetically engineered rats to evaluate PARKIN ...

The brain pathology of Alzheimer’s disease (AD) is characterized by the misfolding and aggregation of both the amyloid beta (Aβ) peptide and hyperphosphorylated forms of the tau protein. Initial Aβ deposition is considered to trigger a sequence of deleteri ...

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 (Mfn2) in NAc neurons ...

Astrocytes play a crucial role in the onset and progression of amyotrophic lateral sclerosis (ALS), a fatal disorder marked by the degeneration of motor neurons (MNs) in the central nervous system. Although astrocytes in ALS are known to be toxic to MNs, t ...

Provided herein are materials and methods for efficiently delivering nucleic acids to cochlear and vestibular cells, and methods of treating sensory transduction disorders associated with a genetic defect. ...

Spinal muscular atrophy (SMA) is a neuromuscular disorder affecting young children. While pre-clinical models of SMA show small spleens, the same is not true in humans. Here, we show by doppler ultrasonography decreased splenic blood flow in Smn(2B/-) mice ...

The invention provides pharmaceutical compositions, combinations and vectors, such as expression vectors, as well as use thereof in a method for central nervous system repair after neurotrauma, such as spinal cord injury (SCI) or stroke and in a method for ...

Bernard Schneider

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The Links between ALS and NF-κB

Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth

Dopamine and Methamphetamine Differentially Affect Electron Transport Chain Complexes and Parkin in Rat Striatum: New Insight into Methamphetamine Neurotoxicity

Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy

Efficient viral transduction in mouse inner ear hair cells with utricle injection and AAV9-PHP.B

Long-term doxycycline-regulated secretion of erythropoietin by encapsulated myoblasts

Single-cell and spatial atlases of spinal cord injury in the Tabulae Paralytica

Parkin regulates drug-taking behavior in rat model of methamphetamine use disorder

Anti-Aβ antibodies bound to neuritic plaques enhance microglia activity and mitigate tau pathology

Production and Purification of Adeno-Associated Viral Vectors (AAVs) Using Orbitally Shaken HEK293 Cells

Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function

TNFα prevents FGF4-mediated rescue of astrocyte dysfunction and reactivity in human ALS models

Compositions and methods for delivering nucleic acids to cochlear and vestibular cells

Allele-specific gene disruption through discrimination of a single base change by S. aureus Cas9-KKH prevents progressive hearing loss after AAV-mediated gene delivery

Blood Flow to the Spleen is Altered in a Mouse Model of Spinal Muscular Atrophy

Neurotrauma treatment

Proton and phosphorus MRS of a 5xFAD mouse model of Alzheimer's disease

Cas9/gRNAs Selective Targeting of the Beethoven Tmc-1 Mutant Allele for Treating Progressive Hearing Loss by AAV-Based Delivery

Proteins associated with the sarcomere and costamere in hearts are dysregulated in two mouse models of spinal muscular atrophy

Encapsulation device