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In this work, mineral oil and synthetic esters were selected at different ageing factors (based on acidity values). Fresh and aged oils have been subjected to high-energy discharges (repeated 100 breakdowns) to simulate electric faults of highly vulnerable intensity. The intent of this work is to understand the influence of high-energy electric faults on oil degradation and gassing tendency at different ageing conditions. In this study, the influence of the high-energy discharges on degradation and gassing tendency at different ageing factors is reported for mineral oil and synthetic esters. Oil degradation is reported by adopting ultraviolet spectroscopy, turbidity and particle counter as per american society for testing and materials (ASTM) standard test methods. Gassing tendencies and fault gas analysis are understood by dissolved the gas analysis using Duval's triangle and Duval's pentagon methods for mineral and non-mineral oils. It is found that the influence of high-energy discharges on oil degradation is higher in mineral oils to that of the synthetic esters. The intensity of the gassing tendency is higher for ester fluids; however, as per the Duval methods, the faulty conditions are at lower levels as compared to mineral oils.

Limb-girdle muscular dystrophies (LGMD) are a heterogeneous group of pathologies. We have identified a cohort of 14 French–Canadian patients from eight different families displaying a novel form of LGMD with an autosomal recessive inheritance. These patients share some features with previously described cases of ‘quadriceps myopathy’ that evolved into an LGMD. All demonstrate quadriceps femoris asymmetrical atrophy. Creatine kinase values were variable from normal to 6000 U/l. Clinical evaluations and MRI studies demonstrate a variable intrafamilial and interfamilial phenotype. Asymmetrical muscle involvement was clinically observed and confirmed by imaging. MRI studies suggest that the hamstrings and the adductor magnus are the first limb muscles to demonstrate fatty infiltration. Muscle pathology shows no sign of active inflammation but increased endomysial connective tissue associated with basal lamina duplication and collagen disorganization. A genome-wide scan using the two largest families uncovered linkage to marker D11S1360 on chromosome 11p12 [multipoint logarithm of the odds (LOD) score of 2.78]. Further genotyping for the eight families confirmed linkage to this new LGMD locus (multipoint LOD score of 4.56). Fine mapping subsequently defined a less than 3.3 cM candidate interval on 11p13–p12. Haplotype analysis of carrier chromosomes suggests that the most frequent mutation may account for up to 81.3% of French–Canadian mutations. In this study, we describe the chromosomal locus of a new form of recessive LGMD with prominent quadriceps femoris atrophy. [ABSTRACT FROM AUTHOR]

The intent of this work is to understand the influence of low energy discharge electric faults in mineral oil and synthetic esters on liquid degradation and gassing tendency at different aging conditions (based on acidity values). A low energy discharge electric fault has been created by continuous discharge of 9 kV for five hours on the liquid surface using a suitable laboratory setup. Liquid degradation is reported by adopting UV spectroscopy, turbidity, and particle counter measurements. The gassing tendency is understood by dissolved gas analysis using Duval’s triangle and Duval’s pentagon methods for mineral oil and non-mineral oils accordingly. It is observed that the influence of low energy discharges on liquid degradation is higher in mineral oils than synthetic esters. The fault gasses in mineral oil are involved with electrical and thermal faults accompanied by stray gassing whereas only partial discharge activity is noticed for synthetic esters. Importantly, the existence of low energy discharge faults like corona discharges will involve a generation of excess high molecular weight products as compared to low molecular weight products that are soluble in liquid volume.

A body of research indicates that people are prone to overestimate the affective impact of future events. Here, we developed a novel experimental paradigm to study these affective forecasting biases under laboratory conditions using subjective (arousal and valence) and autonomic measures (skin conductance responses, SCRs, and heart rate). Thirty participants predicted their emotional responses to 15 unpleasant, 15 neutral, and 15 pleasant scenarios (affective forecasting phase) to which they were then exposed in virtual reality (emotional experience phase). Results showed that participants anticipated more extreme arousal and valence scores than they actually experienced for unpleasant and pleasant scenarios. The emotional experience phase was characterized by classic autonomic patterns, i.e., higher SCRs for emotionally arousing scenarios and greater peak cardiac acceleration for pleasant scenarios. During the affective forecasting phase, we found only a moderate association between arousal scores and SCRs and no valence-dependent modulation of cardiac activity. This paradigm opens up new perspectives for investigating affective forecasting abilities under lab-controlled conditions, notably in psychiatric disorders with anxious anticipations., (© 2023. The Author(s).)

Nano and femtosecond laser writing are becoming very popular techniques for patterning carbon-based materials, as they are single-step processes enabling the drawing of complex shapes without photoresist. However, pulsed laser writing requires costly laser sources and is known to cause damages to the surrounding material. By comparison, continuous-wave lasers are cheap, stable and provide energy at a more moderate rate. Here, we show that a continuous-wave laser may be used to pattern vertical nano-crystalline graphite thin films with very few macroscale defects. Moreover, a spatially resolved study of the impact of the annealing to the crystalline structure and to the oxygen ingress in the film is provided: amorphization, matter removal and high oxygen content at the center of the beam; sp(2) clustering and low oxygen content at its periphery. These data strongly suggest that amorphization and matter removal are controlled by carbon oxidation. The simultaneous occurrence of oxidation and amorphization results in a unique evolution of the Raman spectra as a function of annealing time, with a decrease of the I(D)/I(G) values but an upshift of the G peak frequency.

Hereditary sensory and autonomic neuropathy type 2 (HSAN2) is a rare recessive genetic disorder characterized by severe sensory loss affecting the tactile, thermal and nociceptive modalities. Although heterozygous carriers of nonsense mutations in the HSN2 gene, called with-no-lysine(K)-1 (WNK1), do not develop the disease, historical and experimental evidence suggests that these individuals might perceive somatosensory stimuli differently from others. Using the method-of-limits, we assessed the thresholds for warmth detection, cool detection, heat pain and cold pain in 25 mutation carriers and 35 controls. In group analyses, carriers displayed significantly lower warmth (p<0.001) and cool (p<0.05) difference thresholds, and also tended to report cold pain at higher temperatures (p=0.095), than controls. Similarly, matched-pair analyses showed that carriers are significantly more sensitive to warm stimuli (p<0.01) and cold pain stimuli (p<0.05), and tend to be more sensitive to cool stimuli (p=0.11). Furthermore, the differences between the warmth detection thresholds of the carriers and those of gender- and sex-matched wild types significantly increased with age (r=0.76, p=0.02), and in carriers cool detection thresholds did not increase with age (r=0.27, p=0.24) as expected and observed in controls (r=0.34, p=0.05). This study demonstrates that the carriers of a recessive mutation for HSAN2 display greater sensitivity to innocuous thermal stimuli, as well as for cold pain, suggesting a possible environmental adaptive advantage of the heterozygous state.

Limb-girdle muscular dystrophies (LGMD) are a heterogeneous group of pathologies. We have identified a cohort of 14 French-Canadian patients from eight different families displaying a novel form of LGMD with an autosomal recessive inheritance. These patients share some features with previously described cases of 'quadriceps myopathy' that evolved into an LGMD. All demonstrate quadriceps femoris asymmetrical atrophy. Creatine kinase values were variable from normal to 6000 U/l. Clinical evaluations and MRI studies demonstrate a variable intrafamilial and interfamilial phenotype. Asymmetrical muscle involvement was clinically observed and confirmed by imaging. MRI studies suggest that the hamstrings and the adductor magnus are the first limb muscles to demonstrate fatty infiltration. Muscle pathology shows no sign of active inflammation but increased endomysial connective tissue associated with basal lamina duplication and collagen disorganization. A genome-wide scan using the two largest families uncovered linkage to marker D11S1360 on chromosome 11p12 [multipoint logarithm of the odds (LOD) score of 2.78]. Further genotyping for the eight families confirmed linkage to this new LGMD locus (multipoint LOD score of 4.56). Fine mapping subsequently defined a less than 3.3 cM candidate interval on 11p13-p12. Haplotype analysis of carrier chromosomes suggests that the most frequent mutation may account for up to 81.3% of French-Canadian mutations. In this study, we describe the chromosomal locus of a new form of recessive LGMD with prominent quadriceps femoris atrophy.

Congenital muscular dystrophies (CMDS) are a heterogeneous group of disorders. A growing number of CMDS have been found to be associated with joint hyperlaxity. We recruited 14 French-Canadian cases belonging to 11 families affected by a novel autosomal recessive congenital muscular dystrophy with hyperlaxity (CMDH). All cases come from the southwestern part of Quebec, suggesting a new French-Canadian founder effect. All patients present muscle weakness, proximal contractures coexisting with distal joint hyperlaxity. Pathological and genetic studies have excluded that mutations in the three genes coding for collagen VI subunits are responsible for this disease. A genome-wide scan established linkage of two CMDH families to a region on chromosome 3p23-21. Further linkage analysis confirmed that all families are linked to the same region (log of the odds score of 5.3). Haplotype analysis defines a 1.6-cM candidate interval and suggests that two common mutations may account for 78% of carrier chromosomes. This study describes and maps a new form of recessive CMD with joint hyperlaxity distinct from Ullrich and Bethlem myopathies with a founder effect in the French-Canadian population.