Incorporation of nio into sio2, tio2, al2o3, and na4.2ca2.8(si6o18) matrices: Medium effect on the optical properties and catalytic degradation of methylene blue

The medium effect of the optical and catalytic degradation of methylene blue was studied in the NiO/SiO2, NiO/TiO2, NiO/Al2O3, and NiO/Na4.2Ca2.8(Si6O18) composites, which were prepared by a solid-state method. The new composites were characterized by XRD (X-ray diffraction of powder), SEM/EDS, TEM, and HR-TEM. The size of the NiO nanoparticles obtained from the PSP-4-PVP (polyvinylpyrrolidone) precursors inside the different matrices follow the order of SiO2 > TiO2 > Al2O3 . However, NiO nanoparticles obtained from the chitosan precursor does not present an effect on the particle size. It was found that the medium effect of the matrices (SiO2, TiO2, Al2O3, and Na4.2Ca2.8(Si6O18)) on the photocatalytic methylene blue degradation, can be described as a specific interaction of the NiO material acting as a semiconductor with the MxOy materials through a possible p-n junction. The highest catalytic activity was found for the TiO2 and glass composites where a favorable p-n junction was formed. The isolating character of Al2O3 and SiO2 and their non-semiconductor behavior preclude this interaction to form a p-n junction, and thus a lower catalytic activity. NiO/SiO2 and NiO/Na4.2Ca2.8(Si6O18) showed a similar photocatalytic behavior. On the other hand, the effect of the matrix on the optical properties for the NiO/SiO2, NiO/TiO2, NiO/Al2O3, and NiO/Na4.2Ca2.8(Si6O18) composites can be described by the different dielectric constants of the SiO2, TiO2, Al2O3, Na4.2Ca2.8(Si6O18) matrices. The maxima absorption of the composites (¿max) exhibit a direct relationship with the dielectric constants, while their semiconductor bandgap (Eg) present an inverse relationship with the dielectric constants. A direct relationship between ¿max and Eg was found from these correlations. The effect of the polymer precursor on the particle size can explain some deviations from this relationship, as the correlation between the particle size and absorption is well known. Finally, the NiO/Na4.2Ca2.8(Si6O18) composite was reported in this work for the first time

A fabric-based soft hand exoskeleton for assistance: the ExHand Exoskeleton

INTRODUCTION: The rise of soft robotics has driven the development of devices for assistance in activities of daily living (ADL). Likewise, different types of actuation have been developed for safer human interaction. Recently, textile-based pneumatic actuation has been introduced in hand exoskeletons for features such as biocompatibility, flexibility, and durability. These devices have demonstrated their potential use in assisting ADLs, such as the degrees of freedom assisted, the force exerted, or the inclusion of sensors. However, performing ADLs requires the use of different objects, so exoskeletons must provide the ability to grasp and maintain stable contact with a variety of objects to lead to the successful development of ADLs. Although textile-based exoskeletons have demonstrated significant advancements, the ability of these devices to maintain stable contact with a variety of objects commonly used in ADLs has yet to be fully evaluated. MATERIALS AND METHODS: This paper presents the development and experimental validation in healthy users of a fabric-based soft hand exoskeleton through a grasping performance test using The Anthropomorphic Hand Assessment Protocol (AHAP), which assesses eight types of grasping with 24 objects of different shapes, sizes, textures, weights, and rigidities, and two standardized tests used in the rehabilitation processes of post- stroke patients. RESULTS AND DISCUSSION: A total of 10 healthy users (45.50 ± 14.93 years old) participated in this study. The results indicate that the device can assist in developing ADLs by evaluating the eight types of grasps of the AHAP. A score of 95.76 ± 2.90% out of 100% was obtained for the Maintaining Score, indicating that the ExHand Exoskeleton can maintain stable contact with various daily living objects. In addition, the results of the user satisfaction questionnaire indicated a positive mean score of 4.27 ± 0.34 on a Likert scale ranging from 1 to 5

Phosphorylation does not prompt, nor prevent, the formation of alpha-synuclein toxic species in a rat model of Parkinson's disease

Phosphorylation is involved in numerous neurodegenerative diseases. In particular, alpha-synuclein is extensively phosphorylated in aggregates in patients suffering from synucleinopathies. However, the share of this modification in the events that lead to the conversion of alpha-synuclein to aggregated toxic species needed to be clarified. The rat model that we developed through rAAV2/6-mediated expression of alpha-synuclein demonstrates a correlation between neurodegeneration and formation of small filamentous alpha-synuclein aggregates. A mutation preventing phosphorylation (S129A) significantly increases alpha-synuclein toxicity and leads to enhanced formation of beta-sheet-rich, proteinase K-resistant aggregates, increased affinity for intracellular membranes, a disarrayed network of neurofilaments and enhanced alpha-synuclein nuclear localization. The expression of a mutation mimicking phosphorylation (S129D) does not lead to dopaminergic cell loss. Nevertheless, fewer but larger aggregates are formed, and signals of apoptosis are also activated in rats expressing the phosphorylation-mimicking form of alpha-synuclein. These observations strongly suggest that phosphorylation does not play an active role in the accumulation of cytotoxic pre-inclusion aggregates. Unexpectedly, the study also demonstrates that constitutive expression of phosphorylation-mimicking forms of alpha-synuclein does not protect from neurodegeneration. The role of phosphorylation at Serine 129 in the early phase of Parkinson's disease is examined, which brings new perspective to therapeutic approaches focusing on the modulation of kinases/phosphatases activity to control alpha-synuclein toxicity

Morphological characteristics of motor neurons do not determine their relative susceptibility to degeneration in a mouse model of severe spinal muscular atrophy

Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality, resulting primarily from the degeneration and loss of lower motor neurons. Studies using mouse models of SMA have revealed widespread heterogeneity in the susceptibility of individual motor neurons to neurodegeneration, but the underlying reasons remain unclear. Data from related motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), suggest that morphological properties of motor neurons may regulate susceptibility: in ALS larger motor units innervating fast-twitch muscles degenerate first. We therefore set out to determine whether intrinsic morphological characteristics of motor neurons influenced their relative vulnerability to SMA. Motor neuron vulnerability was mapped across 10 muscle groups in SMA mice. Neither the position of the muscle in the body, nor the fibre type of the muscle innervated, influenced susceptibility. Morphological properties of vulnerable and disease-resistant motor neurons were then determined from single motor units reconstructed in Thy.1-YFP-H mice. None of the parameters we investigated in healthy young adult mice - including motor unit size, motor unit arbor length, branching patterns, motor endplate size, developmental pruning and numbers of terminal Schwann cells at neuromuscular junctions - correlated with vulnerability. We conclude that morphological characteristics of motor neurons are not a major determinant of disease-susceptibility in SMA, in stark contrast to related forms of motor neuron disease such as ALS. This suggests that subtle molecular differences between motor neurons, or extrinsic factors arising from other cell types, are more likely to determine relative susceptibility in SMA

A fabric-based soft hand exoskeleton for assistance: the ExHand Exoskeleton

Introduction: The rise of soft robotics has driven the development of devices for assistance in activities of daily living (ADL). Likewise, different types of actuation have been developed for safer human interaction. Recently, textile-based pneumatic actuation has been introduced in hand exoskeletons for features such as biocompatibility, flexibility, and durability. These devices have demonstrated their potential use in assisting ADLs, such as the degrees of freedom assisted, the force exerted, or the inclusion of sensors. However, performing ADLs requires the use of different objects, so exoskeletons must provide the ability to grasp and maintain stable contact with a variety of objects to lead to the successful development of ADLs. Although textile-based exoskeletons have demonstrated significant advancements, the ability of these devices to maintain stable contact with a variety of objects commonly used in ADLs has yet to be fully evaluated. Materials and methods: This paper presents the development and experimental validation in healthy users of a fabric-based soft hand exoskeleton through a grasping performance test using The Anthropomorphic Hand Assessment Protocol (AHAP), which assesses eight types of grasping with 24 objects of different shapes, sizes, textures, weights, and rigidities, and two standardized tests used in the rehabilitation processes of post- stroke patients. Results and discussion: A total of 10 healthy users (45.50 ± 14.93 years old) participated in this study. The results indicate that the device can assist in developing ADLs by evaluating the eight types of grasps of the AHAP. A score of 95.76 ± 2.90% out of 100% was obtained for the Maintaining Score, indicating that the ExHand Exoskeleton can maintain stable contact with various daily living objects. In addition, the results of the user satisfaction questionnaire indicated a positive mean score of 4.27 ± 0.34 on a Likert scale ranging from 1 to 5

Different atrophy-hypertrophy transcription pathways in muscles affected by severe and mild spinal muscular atrophy

BACKGROUND: Spinal muscular atrophy (SMA) is a neurodegenerative disorder associated with mutations of the survival motor neuron gene SMN and is characterized by muscle weakness and atrophy caused by degeneration of spinal motor neurons. SMN has a role in neurons but its deficiency may have a direct effect on muscle tissue. METHODS: We applied microarray and quantitative real-time PCR to study at transcriptional level the effects of a defective SMN gene in skeletal muscles affected by the two forms of SMA: the most severe type I and the mild type III. RESULTS: The two forms of SMA generated distinct expression signatures: the SMA III muscle transcriptome is close to that found under normal conditions, whereas in SMA I there is strong alteration of gene expression. Genes implicated in signal transduction were up-regulated in SMA III whereas those of energy metabolism and muscle contraction were consistently down-regulated in SMA I. The expression pattern of gene networks involved in atrophy signaling was completed by qRT-PCR, showing that specific pathways are involved, namely IGF/PI3K/Akt, TNF-alpha/p38 MAPK and Ras/ERK pathways. CONCLUSION: Our study suggests a different picture of atrophy pathways in each of the two forms of SMA. In particular, p38 may be the regulator of protein synthesis in SMA I. The SMA III profile appears as the result of the concurrent presence of atrophic and hypertrophic fibers. This more favorable condition might be due to the over-expression of MTOR that, given its role in the activation of protein synthesis, could lead to compensatory hypertrophy in SMA III muscle fibers

Protein 4.1B Contributes to the Organization of Peripheral Myelinated Axons

Neurons are characterized by extremely long axons. This exceptional cell shape is likely to depend on multiple factors including interactions between the cytoskeleton and membrane proteins. In many cell types, members of the protein 4.1 family play an important role in tethering the cortical actin-spectrin cytoskeleton to the plasma membrane. Protein 4.1B is localized in myelinated axons, enriched in paranodal and juxtaparanodal regions, and also all along the internodes, but not at nodes of Ranvier where are localized the voltage-dependent sodium channels responsible for action potential propagation. To shed light on the role of protein 4.1B in the general organization of myelinated peripheral axons, we studied 4.1B knockout mice. These mice displayed a mildly impaired gait and motility. Whereas nodes were unaffected, the distribution of Caspr/paranodin, which anchors 4.1B to the membrane, was disorganized in paranodal regions and its levels were decreased. In juxtaparanodes, the enrichment of Caspr2, which also interacts with 4.1B, and of the associated TAG-1 and Kv1.1, was absent in mutant mice, whereas their levels were unaltered. Ultrastructural abnormalities were observed both at paranodes and juxtaparanodes. Axon calibers were slightly diminished in phrenic nerves and preterminal motor axons were dysmorphic in skeletal muscle. βII spectrin enrichment was decreased along the axolemma. Electrophysiological recordings at 3 post-natal weeks showed the occurrence of spontaneous and evoked repetitive activity indicating neuronal hyperexcitability, without change in conduction velocity. Thus, our results show that in myelinated axons 4.1B contributes to the stabilization of membrane proteins at paranodes, to the clustering of juxtaparanodal proteins, and to the regulation of the internodal axon caliber

Neuromuscular Junction Defects in Mice with Mutation of dynein heavy chain 1

Disruptions in axonal transport have been implicated in a wide range of neurodegenerative diseases. Cramping 1 (Cra1/+) and Legs at odd angles (Loa/+) mice, with hypomorphic mutations in the dynein heavy chain 1 gene, which encodes the ATPase of the retrograde motor protein dynein, were originally reported to exhibit late onset motor neuron disease. Subsequent, conflicting reports suggested that sensory neuron disease without motor neuron loss underlies the phenotypes of Cra1/+ and Loa/+ mice. Here, we present behavioral and anatomical analyses of Cra1/+ mice. We demonstrate that Cra1/+ mice exhibit early onset, stable behavioral deficits, including abnormal hindlimb posturing and decreased grip strength. These deficits do not progress through 24 months of age. No significant loss of primary motor neurons or dorsal root ganglia sensory neurons was observed at ages where the mice exhibited clear symptomatology. Instead, there is a decrease in complexity of neuromuscular junctions. These results indicate that disruption of dynein function in Cra1/+ mice results in abnormal morphology of neuromuscular junctions. The time course of behavioral deficits, as well as the nature of the morphological defects in neuromuscular junctions, suggests that disruption of dynein function in Cra1/+ mice causes a developmental defect in synapse assembly or stabilization

Motor Unit Abnormalities in Dystonia musculorum Mice

Dystonia musculorum (dt) is a mouse inherited sensory neuropathy caused by mutations in the dystonin gene. While the primary pathology lies in the sensory neurons of dt mice, the overt movement disorder suggests motor neurons may also be affected. Here, we report on the contribution of motor neurons to the pathology in dt27J mice. Phenotypic dt27J mice display reduced alpha motor neuron cell number and eccentric alpha motor nuclei in the ventral horn of the lumbar L1 spinal cord region. A dramatic reduction in the total number of motor axons in the ventral root of postnatal day 15 dt27J mice was also evident. Moreover, analysis of the trigeminal nerve of the brainstem showed a 2.4 fold increase in number of degenerating neurons coupled with a decrease in motor neuron number relative to wild type. Aberrant phosphorylation of neurofilaments in the perikaryon region and axonal swellings within the pre-synaptic terminal region of motor neurons were observed. Furthermore, neuromuscular junction staining of dt27J mouse extensor digitorum longus and tibialis anterior muscle fibers showed immature endplates and a significant decrease in axon branching compared to wild type littermates. Muscle atrophy was also observed in dt27J muscle. Ultrastructure analysis revealed amyelinated motor axons in the ventral root of the spinal nerve, suggesting a possible defect in Schwann cells. Finally, behavioral analysis identified defective motor function in dt27J mice. This study reveals neuromuscular defects that likely contribute to the dt27J pathology and identifies a critical role for dystonin outside of sensory neurons