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Ferroelectric single crystals Pb(Zn1/3Nb2/3)O3–PbTiO3 (PZN–PT) are promising full materials for nonresonant or large bandwidth transducers due to the large values of their piezoelectric properties (dij, kij) and their mechanical quality factor (Qij). However the properties of these materials depend greatly on the content of titanium which influences very significantly the symmetry of the crystal. In this paper we try to understand the influence of the percentage of Titanium in these crystals by studying the two compositions that are in very different phases at room temperature (rhombohedral and tetragonal symmetries). Crystals of pure PZN–4.5PT and PZN-12PT were grown by a Flux technique. The typical single crystals obtained are brown yellow. The room temperature dielectric permittivity along the direction is about 900 for the PZN-12PT, which is smaller than that of the PZN–4.5PT (5840). The Curie point Tc of the tetragonal crystal is about 220 °C (which is higher than that of the rhombohedral one (166 °C)), while the ferroelectric phase transition temperature is 130 °C for the PZN-4.5PT single crystal. The remnant polarization and coercive field of oriented crystals measured at 1 kHz are around 40 μC/cm2 and 3.30 kV/cm, respectively for the PZN-4.5PT, 27μC/cm2 and 11.1 kV/cm for the PZN-12PT single crystal. The d33 versus uniaxial stress shows that this coefficient is more stable for the single crystal with low Ti than for that with high rate of Ti. The study of temperature stability on these crystals shows a possible presence of an unidentified phase in the low and negative range temperature for the PZN-4.5PT.

Cellulose nanofibers (CNFs) nanocomposites were prepared using poly(methylmethacrylate-co-methacrylic acid) (PMMA-co-MAA) to investigate the macromolecular mobility within the composite, with particular focus on the effect of H-bonding. Dynamic mechanical analysis (DMA) and broadband dielectric spectroscopy (BDS) were used to fully characterize the molecular mobility for which the effect of the introduction of H-bond forming moieties and the addition of CNFs (5 and 15 wt%) were assessed. Despite similar T g values (determined by Differential Scanning Calorimetry), a deeper analysis of the relaxation times associated with the α-relaxation evidenced a significant effect induced by CNFs, which is in fact slowing down the macromolecular relaxation processes. The activation energy of the β-relaxation remained unchanged despite the introduction of MAA units in the main chain and the successive addition of CNFs. However, the latter led to the appearance at low frequencies of a new β'-relaxation correlated with the interactions between the CNF surface -OH groups and the -COOH groups of the matrix. The γ-relaxation showed a 45 % increase in activation energy from PMMA to PMMA-co-MAA + CNF nanocomposites regardless of the CNF content, due to the possibility of CNFs to interact and hinder the motion of the main chain methyl groups in α position., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Ltd.)

Purpose: To determine the criterion validity of the SET-ULM (Spatial Exploration Test of Upper Limb Mobility), a functional workspace test., Materials and Methods: A prospective study from July 2017 to November 2018 in 30 children with SMA type 1 or 2. All children underwent assessment with the SET-ULM and the Motor Function Measure (MFM)., Results: We included 30 children. Median (Q1; Q3) MFM D1 (standing ability, ambulation and transfers), D2 (axial and proximal motor function), D3 (distal motor function) scores, Total MFM and Total SET-ULM active score were respectively 2.6% (2.6-3.8); 45.8% (19.9-65.3); 57.7% (36.9-80.9); 35.4% (16.7-43.2) and 70.2% (49.7-97.9). Total SET-ULM active score was strongly correlated with the MFM D2 dimension score (rho 0.82; p  < 001), with the D3 dimension (rho 0.86; p  < 0.001) and with the Total MFM score (rho 0.89; p  < 0.005). Total SET-ULM active score differed between SMA types ( p  < 0.01)., Conclusion: The SET-ULM has good criterion validity for the evaluation of available horizontal active upper limb workspace in children with SMA1 and SMA2. Future studies should evaluate reliability and sensitivity to change during a longitudinal follow-up study, as well as in a longitudinal trial of therapeutic effectiveness., Clinical Trials: NCT03223051IMPLICATIONS FOR REHABILITATIONThe Spatial Exploration Test for Upper Limb Mobility is a useful adjunct to the Motor Function Measure.It provides a precise evaluation of horizontal reaching ability.The Spatial Exploration Test for Upper Limb Mobility will be of great clinical utility for the evaluation of the effects of treatments for spinal muscular atrophy.

Poly(methylmethacrylate- co -methacrylic acid) (PMMA- co -MAA) polymers were prepared via cobalt-mediated free radical copolymerization and were characterized after synthesis. The synthesis led to a 98.5% conversion and a final ratio between the two units, MMA/MAA, was equal to 63:37 mol%. PMMA- co -MAA was then used as a matrix for cellulose-based nanocomposites to tailor filler compatibility, thanks to the presence of carboxylic groups capable of generating strong H-bonds with the cellulose surface. Cellulose nanofibers (CNFs) were dispersed using a solution with a mixture of two solvents to tailor compatibility of both the components. For this purpose, CNFs were successfully re-dispersed in methanol using the solvent exchange method and tetrahydrofuran/methanol mixtures at different ratios were used for the preparation of the films. Fully transparent films of PMMA- co -MAA + CNF were prepared up to 15 wt% of CNF with a good dispersion in the matrix. This dispersion state leads to the reinforcement of the polymethacrylate matrix, increasing its tensile strength whilst preserving optical transparency.

Electrothermal processes were studied in pyroresistive composites based on high-density polyethylene (HDPE) containing 8 vol.% carbon black (CB), 8 vol.% carbon fibers (CF), and their mixture 4 vol.% CB + 4 vol.% CF. It is shown that the kinetic heating curves of composites are well described by an exponential dependence with a certain heating rate constant k for each type of composite. After a short heating time, the equilibrium temperature T e is reached in the sample. When the applied voltage exceeds a certain value, the T e value decreases due to the presence of the positive temperature coefficient of resistance (PTC) effect. Due to the PTC effect, the composites exhibit a self-regulating effect relative to the T e . Relations between the applied voltage, electric power, and equilibrium temperature are found, the T e value depends on the applied voltage according to the quadratic law whereas there is a linear relationship between the T e and electric power. A possible application of such pyroresistive composites is resistance welding of plastics using a heating element (HE) made of a pyroresistive material. The use of HDPE-CB composite to create HE for resistance welding is demonstrated and the welded joint of HDPE parts obtained using HE is shown.

Bifidobacteria are saccharolytic bacteria that are able to metabolize a relatively large range of carbohydrates through their unique central carbon metabolism known as the "bifid-shunt". Carbohydrates have been shown to modulate the growth rate of bifidobacteria, but unlike for other genera (e.g., E. coli or L. lactis ), the impact it may have on the overall physiology of the bacteria has not been studied in detail to date. Using glucose and galactose as model substrates in Bifidobacterium longum NCC 2705, we established that the strain displayed fast and slow growth rates on those carbohydrates, respectively. We show that these differential growth conditions are accompanied by global transcriptional changes and adjustments of central carbon fluxes. In addition, when grown on galactose, NCC 2705 cells were significantly smaller, exhibited an expanded capacity to import and metabolized different sugars and displayed an increased acid-stress resistance, a phenotypic signature associated with generalized fitness. We predict that part of the observed adaptation is regulated by the previously described bifidobacterial global transcriptional regulator AraQ, which we propose to reflect a catabolite-repression-like response in B. longum . With this manuscript, we demonstrate that not only growth rate but also various physiological characteristics of B. longum NCC 2705 are responsive to the carbon source used for growth, which is relevant in the context of its lifestyle in the human infant gut where galactose-containing oligosaccharides are prominent.

Nanocomposite films of polybutylene succinate (PBS)/graphene nanoplatelets (GnP) with a GnP content ranging from 0 to 1.35 wt.% were prepared by melt processing. The morphology of both the neat PBS and PBS/GnP nanocomposites were investigated and revealed no significant impact of GnP on the crystalline microstructure. Moisture sorption at 10 °C, 25 °C, and 40 °C were analyzed and modeled using the Guggenheim, Andersen, and De Boer (GAB) equation and Zimm-Lundberg theory, allowing for a phenomenological analysis at the molecular scale. An understanding of the transport sorption properties was proposed by the determination of the molar heat of sorption (Δ H s ), and the activation energy of the diffusion ( E d ) of water in the matrix since both solubility and diffusion are thermo-activable properties. Both Δ H s and E d showed a good correlation with the water clustering theory at high water activity. Water and dioxygen permeabilities ( and ) were determined as a function of temperature and water activity. and decreased with the addition of a small amount of GnP, regardless of the studied temperature. Moreover, the evolution of as a function of water activity was driven by the solubility process, whereas at a given water activity, was driven by the diffusion process. Activation energies of the permeability ( E p ) of water and dioxygen showed a dependency on the nature of the permeant molecule. Finally, from the Δ H s , E d , and E p obtained values, the reduction in water permeability with the addition of a low content of GnP was attributed mainly to a tortuosity effect without diffusive interfaces rather than a significant change in the transport property mechanism.

Fate decisions in the embryo are controlled by a plethora of microenvironmental interactions in a three-dimensional niche. To investigate whether aspects of this microenvironmental complexity can be engineered to direct myogenic human-induced pluripotent stem cell (hiPSC) differentiation, we here screened murine cell types present in the developmental or adult stem cell niche in heterotypic suspension embryoids. We identified embryonic endothelial cells and fibroblasts as highly permissive for myogenic specification of hiPSCs. After two weeks of sequential Wnt and FGF pathway induction, these three-component embryoids are enriched in Pax7-positive embryonic-like myogenic progenitors that can be isolated by flow cytometry. Myogenic differentiation of hiPSCs in heterotypic embryoids relies on a specialized structural microenvironment and depends on MAPK, PI3K/AKT, and Notch signaling. After transplantation in a mouse model of Duchenne muscular dystrophy, embryonic-like myogenic progenitors repopulate the stem cell niche, reactivate after repeated injury, and, compared to adult human myoblasts, display enhanced fusion and lead to increased muscle function. Altogether, we provide a two-week protocol for efficient and scalable suspension-based 3D derivation of Pax7-positive myogenic progenitors from hiPSCs., (© 2022 The Authors.)

Background: MicroRNAs (miRNAs) are small noncoding RNAs involved in posttranscriptional regulation. miRNAs can be secreted and found in many body fluids, and although they are particularly abundant in breastmilk, their functions remain elusive. Human milk (HM) miRNAs start to raise considerable interest, but a comprehensive understanding of the repertoire and expression profiles along lactation has not been well characterized., Objectives: This study aimed to characterize the longitudinal profile of HM miRNA between the second week and third month postpartum., Methods: We used a new sensitive technology to measure HM miRNAs in a cohort of 44 French mothers [mean ± SD age: 31 ± 3.5; BMI (in kg/m2) 21.8 ± 2.3] who delivered at term and provided HM samples at 3 time points (17 ± 3 d, 60 ± 3 d, and 90 ± 3 d) during follow-up visits., Results: We detected 685 miRNAs, of which 35 showed a high and stable expression along the lactation period analyzed. We also described for the first time a set of 11 miRNAs with a dynamic expression profile. To gain insight into the potential functional relevance of this set of miRNAs, we selected miR-3126 and miR-3184 to treat undifferentiated Caco-2 human intestinal cells and then assessed differentially expressed genes and modulation of related biological pathways., Conclusions: Overall, our study provides new insights into HM miRNA composition and, to our knowledge, the first description of its longitudinal dynamics in mothers who delivered at term. Our in vitro results obtained in undifferentiated Caco-2 human intestinal cells transfected with HM miRNAs also provide further support to the hypothesized mother-to-neonate signaling role of HM miRNAs. This trial was registered at clinicaltrials.gov as NCT01894893., (© The Author(s) 2021. Published by Oxford University Press on behalf of the American Society for Nutrition.)

Polymorphisms in genes related to the metabolism of vitamin B12 haven't been examined in a Brazilian population. To (a) determine the correlation between the local genetic ancestry components and vitamin B12 levels using ninety B12-related genes; (b) determine associations between these genes and their SNPs with vitamin B12 levels; (c) determine a polygenic risk score (PRS) using significant variants. This cross-sectional study included 168 children and adolescents, aged 9-13 years old. Total cobalamin was measured in plasma. Genotyping arrays and whole exome data were combined to yield ~ 7000 SNPs in 90 genes related to vitamin B12. The Efficient Local Ancestry Inference was used to estimate local ancestry for African (AFR), Native American, and European (EUR). The association between the genotypes and vitamin B12 levels were determined with generalized estimating equation. Vitamin B12 levels were driven by positive (EUR) and negative (AFR, AMR) correlations with genetic ancestry. A set of 36 variants were used to create a PRS that explained 42% of vitamin level variation. Vitamin B12 levels are influenced by genetic ancestry and a PRS explained almost 50% of the variation in plasma cobalamin in Brazilian children and adolescents.

Polybutylene Succinate (PBS)/Graphene nanoplatelets (GnP) nanocomposites over a range of GnP from 0 to 1.35 wt.%. were prepared by a melt process. A mixture of individual graphene nanosheets and aggregates was obtained by the addition of GnP in the PBS matrix. The presence of these fillers did not significantly modify the morphology, crystalline microstructure of the matrix or its thermal stability. However, a slight reinforcement effect of PBS was reported in the presence of GnP. The water sorption isotherm modelling with Guggenheim, Andersen and De Boer (GAB) equation and Zimm-Lundberg theory allowed a phenomenological analysis at the molecular scale. The presence of GnP did not modify the water sorption capacity of the PBS matrix. From a kinetic point of view, a decrease of the diffusion coefficient with the increasing GnP content was obtained and was attributed to a tortuosity effect. The influence of water activity was discussed over a range of 0.5 to 1 and 0 to 0.9 for water and dioxygen permeability. Improvement of the barrier properties by 38% and 35% for water and dioxygen permeability respectively were obtained.

Introduction: Nusinersen is associated with an improvement in motor function in children with spinal muscular atrophy (SMA) but data on respiratory muscles strength are scarce. Respiratory muscles performance and lung function were evaluated in children with SMA 1c and 2 after six injections of nusinersen (M14). Results from patients with SMA2 were compared with data of age-matched historical controls. Motor function tests (MFM and HINE-2) were assessed at baseline and M14 in the treated patients., Results: Sixteen children (2 SMA Type 1c and 14 SMA Type 2), mean age 9.4 ± 2.3 years, were included. The data of 14 historical SMA 2 controls (mean age 9.3 ± 1.9 years) were gathered. The strength of the global inspiratory muscles of SMA 2 treated with nusinersen, assessed on maximal static inspiratory pressure, forced vital capacity, and esophageal pressure during a maximal sniff was significantly better compared with historical controls (p < .05). A significant improvement in MFM and HINE-2 was observed in the patients with 16 SMA treated with nusinersen after 14 months as compared with baseline., Conclusion: In children with SMA Type 2, respiratory muscle performance was significantly better after six injections of nusinersen as compared with age-matched SMA Type 2 historical controls., (© 2020 Wiley Periodicals LLC.)

The incorporation of graphene into a polymer matrix can endow composites with extended functions. However, it is difficult to well disperse pristine graphene into a polymer matrix in order to obtain polymer nanocomposites due to the lack of functional groups on the surface for bonding with a polymer matrix. Herein, we investigated the role of fluorinated ionic liquid (IL) as a new interfacial agent in poly(vinylidene fluoride-co-chlorotrifluoroethylene) (P(VDF-CTFE))/graphene composite films. First, a task-specific IL, perfluorooctyltriphenylphosphonium iodide (IL-C8F13), was synthesized and adsorbed on the surface of graphene oxide (GO) and reduced graphene oxide (rGO) for making functional nanofillers which were capable of being incorporated into the P(VDF-CTFE) matrix. The cation structure of IL combined three phenyls (potential π-π interactions with graphene) and a short fluorinated chain (enhanced miscibility with fluorinated matrix via dipolar interactions) to make a compatible graphene filler and P(VDF-CTFE) matrix at the interface among them. Second, two series of P(VDF-CTFE)/GO-IL and P(VDF-CTFE)/rGO-IL composites with different loading contents were prepared with the goal of providing an understanding of the mechanism of interfacial interactions. This paper investigated the difference in the interaction model between GO with IL and rGO with IL. Subsequently, the interfacial effect of IL on the properties of P(VDF-CTFE)/graphene composites, such as crystallization, chain segmental relaxation behavior, dispersion, and the final dielectric properties will be further studied.

β-Myrcene (My), a natural 1,3-diene, and isobornyl methacrylate (IBOMA), from partially bio-based raw materials sources, were copolymerized by nitroxide-mediated polymerization (NMP) in bulk using the SG1-based BlocBuilder™ alkoxyamine functionalized with an N -succinimidyl ester group, NHS-BlocBuilder, at T = 100 °C with initial IBOMA molar feed compositions f IBOMA,0 = 0.10-0.90. Copolymer reactivity ratios were r My = 1.90-2.16 and r IBOMA = 0.02-0.07 using Fineman-Ross, Kelen-Tudos and non-linear least-squares fitting to the Mayo-Lewis terminal model and indicated the possibility of gradient My/IBOMA copolymers. A linear increase in molecular weight versus P NMR analysis. The preparation by NMP of My/IBOMA thermoplastic elastomers (TPEs), mostly bio-sourced, was then attempted. IBOMA-My-IBOMA triblock copolymers containing a minor fraction of My or styrene (S) units in the outer hard segments ( Đ ≤ 1.41) were exhibited by My/IBOMA copolymerization with f IBOMA,0 ≤ 0.80. My-rich and IBOMA-rich copolymers were shown to have a high degree of chain-end fidelity by performing subsequent chain-extensions with IBOMA and/or My, and by 31 P NMR analysis. The preparation by NMP of My/IBOMA thermoplastic elastomers (TPEs), mostly bio-sourced, was then attempted. IBOMA-My-IBOMA triblock copolymers containing a minor fraction of My or styrene (S) units in the outer hard segments ( M n s at about -60 °C and +180 °C and confirmed by atomic force microscopy (AFM). A plastic stress-strain behavior (stress at break -1 , Đ = 1.91-2.23 and F IBOMA = 0.28-0.36) were synthesized using SG1-terminated poly(ethylene- stat -butylene) dialkoxyamine. The micro-phase separation was suggested by the detection of two distinct T g s at about -60 °C and +180 °C and confirmed by atomic force microscopy (AFM). A plastic stress-strain behavior (stress at break σ B = 3.90 ± 0.22 MPa, elongation at break ε B = 490 ± 31%) associated to an upper service temperature of about 140 °C were also highlighted for these triblock polymers., Competing Interests: There are no conflicts to declare., (This journal is © The Royal Society of Chemistry.)

In this work, phosphonium ionic liquids (ILs) based on tetra-alkylphosphonium cations combined with carboxylate, phosphate and phosphinate anions, were used for organic modification of layered double hydroxide (LDH). Two different amounts (2 and 5 wt %) of the organically modified LDHs were mixed with poly(butylene adipate- co -terephthalate) (PBAT) matrix by melt extrusion. All prepared PBAT/IL-modified-LDH composites exhibited increased mechanical properties (20-50% Young's modulus increase), decreased water vapor permeability (30-50% permeability coefficient reduction), and slight decreased crystallinity (10-30%) compared to the neat PBAT., Competing Interests: The authors declare no conflict of interest.

Chitin-silica hybrid thin films, prepared through the colloidal self-assembly of chitin nanorods and siloxane oligomers, have been studied for the first time by PeakForce QNM AFM mode to explore their structure and mechanical behaviour. The change in structure and mechanical properties of chitin-silica hybrids is mainly driven by the relative quantities in chitin nanorods and silica, expressed as the chitin volume fraction ϕ chi . The coating of the chitin polysaccharide by silica leads to an increase of the nanorods diameter and films surface roughness at small ϕ chi values. The DMT (Derjaguin-Muller-Toporov) modulus increased both at small ϕ chi due to a large amount of silica and at very high ϕ chi →1 due to an incomplete tip penetration between nanorods. The local parallel orientation of nanorods observed at different ϕ chi values resulted in a modulus increase due to an enhancement of the cohesion between nanorods., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

PeakForce Quantitative Nanomechanical Mapping (QNM) AFM mode was used to explore the mechanical properties of textured chitin-silica hybrid films at the nanoscale. The influence of the force applied by the tip on the sample surface was studied for standard homogeneous samples, for chitin nanorods and for chitin-silica hybrid nanocomposites. Thick films of superimposed chitin nanorods showed a monotonous increase of DMT modulus (based on the Derjaguin-Muller-Toporov model) owing to an increase in modulus at the interface between nanorods due to geometrical constraints of the AFM acquisition. A similar variation of DMT modulus was obtained for chitin-silica hybrid thick films related to mechanical strengthening induced by the presence of silica. This work revealed the role of the organic-inorganic interface, at the nanoscale, in the mechanical behaviour of textured materials using PeakForce QNM mode, with optimized analysis conditions., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Alport syndrome is an inherited nephropathy associated with mutations in genes encoding type IV collagen chains present in the glomerular basement membrane. COL4A5 mutations are associated with the major X-linked form of the disease, and COL4A3 and COL4A4 mutations are associated with autosomal recessive and dominant forms (thought to be involved in 15% and 1%-5% of the families, respectively) and benign familial hematuria. Mutation screening of these three large genes is time-consuming and expensive. Here, we carried out a combination of multiplex PCR, amplicon quantification, and next generation sequencing (NGS) analysis of three genes in 101 unrelated patients. We identified 88 mutations and 6 variations of unknown significance on 116 alleles in 83 patients. Two additional indel mutations were found only by secondary Sanger sequencing, but they were easily identified retrospectively with the web-based sequence visualization tool Integrative Genomics Viewer. Altogether, 75 mutations were novel. Sequencing the three genes simultaneously was particularly advantageous as the mode of inheritance could not be determined with certainty in many instances. The proportion of mutations in COL4A3 and COL4A4 was notably high, and the autosomal dominant forms of Alport syndrome appear more frequently than reported previously. Finally, this approach allowed the identification of large COL4A3 and COL4A4 rearrangements not described previously. We conclude that NGS is efficient, reduces screening time and cost, and facilitates the provision of appropriate genetic counseling in Alport syndrome., (Copyright © 2014 by the American Society of Nephrology.)