gebhart/patrepeval huggingface dataset:
Dysferlin maintains the integrity of transverse tubules and the junctional sarcoplasmic reticulum following contraction‐induced injury (2012)

Background Mutations in the DYSF gene that encodes the protein, dysferlin, lead to human muscular dystrophies known as dysferlinopathies. Hypothesis We tested the hypothesis that dysferlin is present at transverse tubules (TT) and that it helps stabilize TT and their interaction with the junctional sarcoplasmic reticulum (j‐SR) following contraction‐induced injury in vivo. Methods and Results Through improved tissue fixation and antigen unmasking, we found that most of the dysferlin in skeletal muscle is present in a myoplasmic reticulum that flanks Z‐disks in a pattern similar to markers of TT in healthy rat, mouse, and human skeletal muscle. Dysferlin is enriched in TT and SR fractions in sucrose gradients of rat muscle. Injury by large‐strain lengthening contractions increased the labeling intensity of myoplasmic dysferlin in wild type A/WySnJ mouse muscle. At 3hr post‐injury, dysferlin‐null A/J mouse muscle showed disrupted labeling for TT and j‐SR markers in ~75% fibers, whereas only ~20% showed disruption in wild type muscle. By 72hr post‐injury, ~20% and ~2% fibers were necrotic in A/J and A/WySnJ muscle, respectively, and the number of necrotic fibers was reduced 5‐fold in A/J mice treated with the L‐type calcium channel blocker, diltiazem. Conclusion Dysferlin plays an important role in protecting TT and the j‐SR from damage following contraction‐induced injury.
Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: association with neuromyelitis optica (2009)

This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.
Discovery of first-in-class nanomolar inhibitors of heptosyltransferase I reveals a new aminoglycoside target and potential alternative mechanism of action (2022)

A clinically relevant inhibitor for Heptosyltransferase I (HepI) has been sought after for many years because of its critical role in the biosynthesis of lipopolysaccharides on bacterial cell surfaces. While many labs have discovered or designed novel small molecule inhibitors, these compounds lacked the bioavailability and potency necessary for therapeutic use. Extensive characterization of the HepI protein has provided valuable insight into the dynamic motions necessary for catalysis that could be targeted for inhibition. Structural inspection of Kdo<sub>2</sub>-lipid A suggested aminoglycoside antibiotics as potential inhibitors for HepI. Multiple aminoglycosides have been experimentally validated to be first-in-class nanomolar inhibitors of HepI, with the best inhibitor demonstrating a K<sub>i</sub> of 600 ± 90 nM. Detailed kinetic analyses were performed to determine the mechanism of inhibition while circular dichroism spectroscopy, intrinsic tryptophan fluorescence, docking, and molecular dynamics simulations were used to corroborate kinetic experimental findings. While aminoglycosides have long been described as potent antibiotics targeting bacterial ribosomes' protein synthesis leading to disruption of the stability of bacterial cell membranes, more recently researchers have shown that they only modestly impact protein production. Our research suggests an alternative and novel mechanism of action of aminoglycosides in the inhibition of HepI, which directly leads to modification of LPS production in vivo. This finding could change our understanding of how aminoglycoside antibiotics function, with interruption of LPS biosynthesis being an additional and important mechanism of aminoglycoside action. Further research to discern the microbiological impact of aminoglycosides on cells is warranted, as inhibition of the ribosome may not be the sole and primary mechanism of action. The inhibition of HepI by aminoglycosides may dramatically alter strategies to modify the structure of aminoglycosides to improve the efficacy in fighting bacterial infections.
Efficient Approach for Angular Modelling of Electrical Machine by Reluctance Network (2013)

International audience
Steam dealkylation of aromatic hydrocarbons. II. Role of the support and kinetic pathway of oxygenated species in toluene steam dealkylation over group VIII metal catalysts. [Catalyst support comparison] (1982)

The role of support in steam dealkylation (SDA) is studied on a series of Group VIII metal catalysts supported on alumina, silica, and titania. When possible turnover frequencies are given on the basis of the free metal fraction during the reaction, the values are generally constant with time-on-steam and represent the actual turnover frequency of the catalysts. Metals can be classified into two groups, namely, support-sensitive metals (Pt,Rh,Pd) and support-insensitive metals (Ni,Co,Ru and to a certain extent Ir). Support sensitivity is related to the oxidizability of the metallic surface. For metals of the first group, the reaction is probably governed by a noncompetitive mechanism in which the metal coverage by the oxygenated species is negligible. Kinetic derivation leads to a rate law where there is at once intervention of the support site concentration and of the specific perimeter of the metal/support interface. One can thus explain the support effect for this metal group and the slight sensitivity to the crystallite size observed in the Rh/Al/sub 2/O/sub 3/ series. For metals of the second group, a competitive mechanism probably takes place on the metal. Kinetic derivation leads to a rate law independent of the support site concentration and accounting for themore » slight negative order with respect to toluene as previously reported. The conspicuous parallelism between the selectivities of the various metals in SDA, in hydrodealkylation , and in hydrogenolysis is also discussed. In addition to the metal, the support and the crystallite size are determining factors of the selectivity to benzene in SDA.« less
Interaction of Heavy-Ion Beams with a Hydrogen Plasma: Plasma Lens Effect and Stopping Power Enhancement (1988)

By coupling a hydrogen plasma to a Tandem accelerator, transmission and energy losses of 2 MeV/u carbon and sulfur beams passing through a plasma target have been investigated. Fluctuations in beam transmission have been observed and attributed to a plasma lens effect. Moreover, energy loss measurements indicate an enhanced stopping power of the plasma relative to its cold matter equivalent.
Energy calibration issues in nuclear resonant vibrational spectroscopy: observing small spectral shifts and making fast calibrations (2013)

The conventional energy calibration for nuclear resonant vibrational spectroscopy (NRVS) is usually long. Meanwhile, taking NRVS samples out of the cryostat increases the chance of sample damage, which makes it impossible to carry out an energy calibration during one NRVS measurement. In this study, by manipulating the 14.4 keV beam through the main measurement chamber without moving out the NRVS sample, two alternative calibration procedures have been proposed and established: (i) an in situ calibration procedure, which measures the main NRVS sample at stage A and the calibration sample at stage B simultaneously, and calibrates the energies for observing extremely small spectral shifts; for example, the 0.3 meV energy shift between the 100%-(57)Fe-enriched [Fe4S4Cl4](=) and 10%-(57)Fe and 90%-(54)Fe labeled [Fe4S4Cl4](=) has been well resolved; (ii) a quick-switching energy calibration procedure, which reduces each calibration time from 3-4 h to about 30 min. Although the quick-switching calibration is not in situ, it is suitable for normal NRVS measurements.
The REQUITE Project: Validating predictive models and biomarkers of radiotherapy toxicity to reduce side-effects and improve quality of life in cancer survivors. (2016)

85 Background: To present the REQUITE project (Validating Predictive Models and Biomarkers of Radiotherapy (RT) toxicity to reduce side-effects and improve quality-of-life in cancer survivors) and first results on the enrollment and acute toxicity (tox) of prostate cancer (Pca) patients (pts). Methods: The European Union funded REQUITE project involves centers in Europe and the USA. The main objectives of the project are to: carry out a multicentre, cohort study collecting blood samples, standardized epidemiology and treatment data, longitudinal side-effect and quality of life data (before and after treatment, years 1 and 2); produce a centralized database and biobank of DNA for 5,300 patients; validate clinical/dosimetric predictors of RT tox and incorporate biomarker data. The project focuses on cancers of the breast, lung and prostate. For PCa the primary endpoint is rectal bleeding at 2 years. Results: A centralized database was established for data collection, including storage of complete dosimetric information in DICOM RT format. Enrollment started in April 2014 and will end in August 2018. In the first 16 months 909 PCa pts were enrolled: 10% low risk, 33% intermediate and 57% high risk. 63% pts received exclusive RT (neoadjuvant hormone therapy in 62%) and 37% post-prostatectomy; 97% external beam RT (prescription doses: 60-85Gy, 2Gy equivalent, alpha/beta=3Gy), while 3% received brachytherapy. Data on acute tox at RT end (measured by CTCAE 4.03) are available for 496 pts. 79 G2-G3 GI tox were reported, with proctitis and diarrhea having the higher prevalence (45 and 30 events respectively). 74 pts exhibited G2-G3 GU tox, with 34 pts reporting multiple symptoms and frequency and urgency having the higher prevalence (43 and 30 events respectively, 25 pts both). Conclusions: REQUITE is proving the feasibility of a prospective standardized collection of epidemiological/clinical/dosimetric/toxicity data coupled to centralized storing of biological material. Meanwhile a large database is being created, which will be of value also for future research in the field of radioinduced side effects. REQUITE is funded by a European Union FP7 grant (601826).
Ultra-stable, Solution-Processable CsPbBr3-SiO2 Nanospheres for Highly Efficient Color Conversion in Micro Light-Emitting Diodes (2022)

Micro light-emitting diodes (μ-LEDs) coupled to color conversion phosphors are among the most promising technologies for future display and artificial light sources. However, current emitters suffer from excessively large particle sizes, preventing micron-scale processability, and/or low stability that hampers the device lifetime. Here, we demonstrate down-conversion μ-LED phosphors based on CsPbBr3 perovskite nanocrystals directly grown inside perfectly sealed mesoporous silica nanospheres (NSs). Key for this advancement is a high-throughput calcination procedure in the presence of K2CO3 as selective pore sealing agent, which simultaneously produces the CsPbBr3 nanocrystals, boosts their emission efficiency to &gt;87%, and perfectly isolates them from the outer environment without causing inter-particle cross-linking or aggregation. This results in size-homogeneous, finely solution-dispersible, ultra-stable, and highly emissive CsPbBr3-SiO2 NSs that fit the technological requirements of photolithographic inks for highly uniform μ-LED color conversion patterns with pixels smaller than 20 μm.
Effects of Point Mutations in the Major Capsid Protein of Beet Western Yellows Virus on Capsid Formation, Virus Accumulation, and Aphid Transmission (2003)

Point mutations were introduced into the major capsid protein (P3) of cloned infectious cDNA of the polerovirus beet western yellows virus (BWYV) by manipulation of cloned infectious cDNA. Seven mutations targeted sites on the S domain predicted to lie on the capsid surface. An eighth mutation eliminated two arginine residues in the R domain, which is thought to extend into the capsid interior. The effects of the mutations on virus capsid formation, virus accumulation in protoplasts and plants, and aphid transmission were tested. All of the mutants replicated in protoplasts. The S-domain mutant W166R failed to protect viral RNA from RNase attack, suggesting that this particular mutation interfered with stable capsid formation. The R-domain mutant R7A/R8A protected approximately 90% of the viral RNA strand from RNase, suggesting that lower positive-charge density in the mutant capsid interior interfered with stable packaging of the complete strand into virions. Neither of these mutants systemically infected plants. The six remaining mutants properly packaged viral RNA and could invade Nicotiana clevelandii systemically following agroinfection. Mutant Q121E/N122D was poorly transmitted by aphids, implicating one or both targeted residues in virus-vector interactions. Successful transmission of mutant D172N was accompanied either by reversion to the wild type or by appearance of a second-site mutation, N137D. This finding indicates that D172 is also important for transmission but that the D172N transmission defect can be compensated for by a "reverse" substitution at another site. The results have been used to evaluate possible structural models for the BWYV capsid.
The amyloid-β degradation intermediate Aβ34 is pericyte-associated and reduced in brain capillaries of patients with Alzheimer’s disease (2019)

An impairment of amyloid β-peptide (Aβ) clearance is suggested to play a key role in the pathogenesis of sporadic Alzheimer's disease (AD). Amyloid degradation is mediated by various mechanisms including fragmentation by enzymes like neprilysin, matrix metalloproteinases (MMPs) and a recently identified amyloidolytic activity of β-site amyloid precursor protein cleaving enzyme 1 (BACE1). BACE1 cleavage of Aβ40 and Aβ42 results in the formation of a common Aβ34 intermediate which was found elevated in cerebrospinal fluid levels of patients at the earliest disease stages. To further investigate the role of Aβ34 as a marker for amyloid clearance in AD, we performed a systematic and comprehensive analysis of Aβ34 immunoreactivity in hippocampal and cortical post-mortem brain tissue from AD patients and non-demented elderly individuals. In early Braak stages, Aβ34 was predominantly detectable in a subset of brain capillaries associated with pericytes, while in later disease stages, in clinically diagnosed AD, this pericyte-associated Aβ34 immunoreactivity was largely lost. Aβ34 was also detected in isolated human cortical microvessels associated with brain pericytes and its levels correlated with Aβ40, but not with Aβ42 levels. Moreover, a significantly decreased Aβ34/Aβ40 ratio was observed in microvessels from AD patients in comparison to non-demented controls suggesting a reduced proteolytic degradation of Aβ40 to Aβ34 in AD. In line with the hypothesis that pericytes at the neurovascular unit are major producers of Aβ34, biochemical studies in cultured human primary pericytes revealed a time and dose dependent increase of Aβ34 levels upon treatment with recombinant Aβ40 peptides while Aβ34 production was impaired when Aβ40 uptake was reduced or BACE1 activity was inhibited. Collectively, our findings indicate that Aβ34 is generated by a novel BACE1-mediated Aβ clearance pathway in pericytes of brain capillaries. As amyloid clearance is significantly reduced in AD, impairment of this pathway might be a major driver of the pathogenesis in sporadic AD.
Optimal control for dynamic grid connected photovoltaic system based on Markov chain (2007)

A dynamic grid-connected photovoltaic system with storage batteries has the characteristics of dynamic, time-varying, random and indetermination. A Markov chain model of the load demand power was built based on Markov decision theory according to the 6kW PV system operating data. The object function of the battery discharge power and one step transition matrix of the load demand power have been obtained After considering the conditions of the dynamic grid-connected photovoltaic system, an optimal control method of the adaptive power distribute was directly computed. The results show the model and algorithm are correct and effective.
Development and Characterization of Composites Consisting of Calcium Phosphate Cements and Mesoporous Bioactive Glass for Extrusion-Based Fabrication (2019)

Calcium phosphate cements (CPC) and mesoporous bioactive glasses (MBG) are two degradable biomaterial groups widely under investigation concerning their applicability to treat bone defects. MBG-CPC composites were recently shown to possess enhanced degradation properties in comparison to pure CPC. In addition, modification of MBG allows an easy incorporation of therapeutically effective ions. Additive manufacturing of such composites enables the fabrication of patient-specific geometries with further improved degradation behavior due to control over macroporosity. In this study, we developed composites prepared from a non-aqueous carrier-liquid (cl) based CPC paste and MBG particles suitable for extrusion-based additive manufacturing (3D plotting). CPC with the addition of up to 10 wt % MBG were processible by adjusting the amount of cl. Scaffolds consisting of a 4, 6 and 8%-MBG-CPC composite were successfully manufactured by 3D plotting. While mechanically characterization of the scaffolds showed an influence of the MBG, no changes of microstructure were observed. During degradation of the composite, the release of Ca<sup>2+</sup> and Sr<sup>2+</sup> ions could be controlled by the MBG composition and plotted scaffolds with macropores showed a significant higher release than bulk samples of comparable mass. These findings demonstrate a high flexibility regarding ion release of the developed composites and suggest utilizing the drug binding capacities of MBG as a prospective delivery system for biologically active proteins.
Origins of low-angle stratification in aeolian deposits (2020)

A significant portion of the aeolian rock record is not high-angle cross-strata, but rather consists of low-angle strata (≤15 degrees). Possible origins for these strata include interdune, sand-sheet, and apron and plinth deposits. Ideally these deposits are distinct, but preservation potential and conflicting models of bedform migration plus similarities in processes complicate interpretations. The geometries of interdune deposits are dune-specific. For transverse crescentic dunes, lensoidal interdune bodies occur climbing in the transport direction. Linear dunes, if envisioned as extending downwind or vertically accreting, produce interdune lenses elongate with the dunes. Recent work suggesting that linear dunes migrate laterally, however, suggests extensive, climbing, sheet-like interdune deposits. Similarly, stationary and vertically accreting star dunes give rise to thick, irregular interdune lenses. In contrast, a net lateral migration for star dunes results in climbing sheets of interdune deposits. Interdune deposits, ideally overlying first-order bounding surfaces and alternating vertically with dune deposits, can also occur amalgamated when a very low angle of climb occurs or can be confused with extra-erg deposits that overlie super bounding surfaces. Sand sheets typically occur on the margins of ergs and their deposits mark the basal, upper or lateral fringe portions of aeolian units. Regional extensiveness and a lack of symmetrical sequences of structures can distinguish sand-sheet deposits from some interdune accumulations. Deposits of dune aprons and lower plinths may occur as the sole record where low net preservation occurs. Plinths and aprons of wind-ripple laminae are best developed on oblique crescentic, linear and star dunes where along-slope secondary airflow is significant. These dunes may leave a record of truncating subsets of low-angle wind-ripple laminae that show a distinct parallelism within subsets.
Quantification of biogenic carbon in fuel blends through LSC 14C direct measurement and assessment of uncertainty (2022)

Biogenic carbon in a fuel blend may be quantified by determining its radiocarbon (14C) content, which is most accurately measured using an accelerator mass spectrometer (AMS). An AMS is a highly specialized instrument requiring significant sample preparation and it is not readily available as a tool for rapid, in-house fuel assessment. Direct fuel measurement by liquid scintillation counter (LSC) analysis is an alternative 14C-based technique that requires only limited expertise and can be performed with instrumentation that can be purchased and installed in-house. Although LSC direct measurement is not an accepted standard method for fuel analysis, it has shown promising results. Here, we further describe the use of 14C liquid scintillation for direct-measurement and quantification of fossil fuel-biofuel blend levels and assess uncertainty. Our study includes analysis of four biofuel types (HT processed gasoline, jet fuel, diesel, and commercial bio-diesel) each blended at 0, 1, 2, 3, 5, and 10% biofuel (V/V) by addition of fossil toluene. Additionally, replicate commercial bio-diesel samples blended at 1% biofuel (V/V) were prepared to verify reproducibility. Each sample was counted for repeated 4-hour periods for a total of 24–64 h to quantify uncertainty as a function of counting time. For all samples, fuel count rate and sample counting efficiency were the dominant sources of error but compete and must be optimized. With increasing biofuel content and sample volume, the sample activity increases but at the expense of decreasing counting efficiency. For a 1% fuel blend level and sample size of 5 mL, a target uncertainty of 0.5% biogenic carbon (C/C, 95% confidence interval) absolute precision is achieved in less than 8 hours counting, and the required counting time decreases to 4 h with a sample size of 10 mL.
Suppression of self-pulsing behavior in erbium-doped fiber lasers with a semiconductor optical amplifier (2002)

We experimentally demonstrate that the stability of cw and mode-locked erbium-doped fiber ring lasers can be improved significantly with a semiconductor optical amplifier (SOA) inside the cavity. The fast saturable gain of the SOA suppresses significantly the self-pulsing that is due to ion pairs in the erbium-doped fiber, which acts as a saturable absorber. A linear stabilization analysis of the laser system agrees with our experimental results.
Complement factor 5 deficiency leads to defective insulin receptor processing and severe systemic insulin resistance (2015)

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
A system for measuring elastic wave velocity under high pressure and high temperature using a combination of ultrasonic measurement and the multi-anvil apparatus at SPring-8 (2009)

An experimental system to measure the elastic wave velocities of hot-pressed polycrystalline samples at high pressure and high temperature has been installed at SPring-8. It uses a combination of the ultrasonic pulse-echo-overlap method and Kawai-type multi-anvil apparatus (SPEED-1500). X-ray radiographic imaging enables the sample length to be determined at high pressure and high temperature, which is indispensable for precise determination of elastic wave velocity. Precise measurements of the elastic wave velocities of various minerals have been determined at pressures up to 19 GPa and temperatures up to 1673 K. The experimental technique provides the precise elastic wave velocities of various materials corresponding to those in the Earth's mantle.