The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements.

Background: Systemic sclerosis-associated interstitial lung disease (ILD) carries a high mortality risk; expert guidance is required to aid early recognition and treatment. We aimed to develop the first expert consensus and define an algorithm for the identification and management of the condition through application of well established methods.

Methods: Evidence-based consensus statements for systemic sclerosis-associated ILD management were established for six domains (ie, risk factors, screening, diagnosis and severity assessment, treatment initiation and options, disease progression, and treatment escalation) using a modified Delphi process based on a systematic literature analysis.

Association between clinical specialty setting and disease management in patients with psoriatic arthritis: results from LOOP, a cross-sectional, multi-country, observational study.

Background: Psoriatic arthritis (PsA) is a chronic and debilitating disease that can be managed by different clinical specialists.

Objectives: The objective of the LOOP study was to evaluate the impact of clinical specialty setting on the time to diagnosis and treatment of patients with PsA. Clinical disease activity and disease burden were also compared between clinical settings.

Clinical and Genetic Features in a Series of Eight Unrelated Patients with Neuropathy Due to Glycyl-tRNA Synthetase (GARS) Variants.

Pathogenic variants in the Glycyl-tRNA synthetase gene cause the allelic disorders Charcot-Marie-Tooth disease type 2D and distal hereditary motor neuropathy type V. We describe clinical features in 8 unrelated patients found to have Glycyl-tRNA synthetase variants by Next Generation Sequencing. In addition to upper limb predominant symptoms, other presentations included failure to thrive, feeding difficulties and lower limb dominant symptoms.

Single-cell adhesion force kinetics of cell populations from combined label-free optical biosensor and robotic fluidic force microscopy.

Single-cell adhesion force plays a crucial role in biological sciences, however its in-depth investigation is hindered by the extremely low throughput and the lack of temporal resolution of present techniques. While atomic force microcopy (AFM) based methods are capable of directly measuring the detachment force values between individual cells and a substrate, their throughput is limited to few cells per day, and cannot provide the kinetic evaluation of the adhesion force over the timescale of several hours. In this study a high spatial and temporal resolution resonant waveguide grating based label-free optical biosensor was combined with robotic fluidic force microscopy to monitor the adhesion of living cancer cells.

Vibrational Stark Spectroscopy of Fluorobenzene Using Quantum Cascade Laser Dual Frequency Combs.

We demonstrate the performance of a dual frequency comb quantum cascade laser (QCL) spectrometer for the application of vibrational Stark spectroscopy. Measurements performed on fluorobenzene with the dual-comb spectrometer (DCS) were compared to results obtained using a conventional Fourier transform infrared (FT-IR) instrument in terms of spectral response, parameter estimation, and signal-to-noise ratio (S/N). The dual-comb spectrometer provided similar qualitative and quantitative data as the FT-IR setup in 250 times shorter acquisition time.

Oxidization increases the binding of EGCG to serum albumin revealed by kinetic data from label-free optical biosensor with reference channel.

Epigallocatechin-gallate (EGCG) is the main polyphenol ingredient of green tea. This compound is a strong antioxidant and oxidizes easily. Numerous studies demonstrated its beneficial effects on the human health, for example its anticancer and anti-inflammatory activity.

Publisher Correction: Spring constant and sensitivity calibration of FluidFM micropipette cantilevers for force spectroscopy measurements.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Internet addiction and functional brain networks: task-related fMRI study.

A common brain-related feature of addictions is the altered function of higher-order brain networks. Growing evidence suggests that Internet-related addictions are also associated with breakdown of functional brain networks. Taking into consideration the limited number of studies used in previous studies in Internet addiction (IA), our aim was to investigate the functional correlates of IA in the default mode network (DMN) and in the inhibitory control network (ICN).

Dysregulation of Mitochondrial Ca Uptake and Sarcolemma Repair Underlie Muscle Weakness and Wasting in Patients and Mice Lacking MICU1.

Muscle function is regulated by Ca, which mediates excitation-contraction coupling, energy metabolism, adaptation to exercise, and sarcolemmal repair. Several of these actions rely on Ca delivery to the mitochondrial matrix via the mitochondrial Ca uniporter, the pore of which is formed by mitochondrial calcium uniporter (MCU). MCU's gatekeeping and cooperative activation are controlled by MICU1.

Impact of demography on linked selection in two outcrossing Brassicaceae species.

Genetic diversity is shaped by mutation, genetic drift, gene flow, recombination, and selection. The dynamics and interactions of these forces shape genetic diversity across different parts of the genome, between populations and species. Here, we have studied the effects of linked selection on nucleotide diversity in outcrossing populations of two Brassicaceae species, and , with contrasting demographic history.

Congenital myasthenic syndrome with mild intellectual disability caused by a recurrent SLC25A1 variant.

Congenital myasthenic syndromes (CMS) are a clinically and genetically heterogeneous group of disorders caused by mutations which lead to impaired neuromuscular transmission. SLC25A1 encodes a mitochondrial citrate carrier, associated mainly with the severe neurometabolic disease combined D-2- and L-2-hydroxyglutaric aciduria (D/L-2-HGA). We previously reported a single family with a homozygous missense variant in SLC25A1 with a phenotype restricted to relatively mild CMS with intellectual disability, but to date no additional cases of this CMS subtype had been reported.

Correction to "High-Resolution Adhesion Kinetics of EGCG-Exposed Tumor Cells on Biomimetic Interfaces: Comparative Monitoring of Cell Viability Using Label-Free Biosensor and Classic End-Point Assays".

[This corrects the article DOI: 10.1021/acsomega.7b01902.

The allelochemical tannic acid affects the locomotion and feeding behaviour of the pond snail, Lymnaea stagnalis, by inhibiting peripheral pathways.

(1) The effect of tannic acid (TA), a dominant component of plant allelochemicals, was investigated on the locomotion and feeding of the pond snail, Lymnaea stagnalis. The effect of TA on the neuronal background underlying feeding activity was also analysed. (2) TA affected the spontaneous locomotion and of juvenile snails in a concentration-dependent way.

Molecular genetic investigations identify new clinical phenotypes associated with BCS1L-related mitochondrial disease.

BCS1L encodes a homolog of the Saccharomyces cerevisiae bcs1 protein, which has a known role in the assembly of Complex III of the mitochondrial respiratory chain. Phenotypes reported in association with pathogenic BCS1L variants include growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis and early death (GRACILE syndrome), and Björnstad syndrome, characterized by abnormal flattening and twisting of hair shafts (pili torti) and hearing problems. Here we describe two patients harbouring biallelic variants in BCS1L; the first with a heterozygous variant c.

Modulation of Agrin and RhoA Pathways Ameliorates Movement Defects and Synapse Morphology in MYO9A-Depleted Zebrafish.

Congenital myasthenic syndromes (CMS) are a group of rare, inherited disorders characterised by impaired function of the neuromuscular junction (NMJ). This is due to defects in one of the many proteins associated with the NMJ. In three patients with CMS, missense mutations in a gene encoding an unconventional myosin protein, MYO9A, were identified as likely causing their disorder.

Adhesion force measurements on functionalized microbeads: An in-depth comparison of computer controlled micropipette and fluidic force microscopy.

Characterization of the binding of functionalized microparticles to surfaces with a specific chemistry sheds light on molecular scale interactions. Polymer or protein adsorption are often monitored by colloid particle deposition. Force measurements on microbeads by atomic force microscopy (AFM) or optical tweezers are standard methods in molecular biophysics, but typically have low throughput.

Clinical presentation and proteomic signature of patients with TANGO2 mutations.

Transport And Golgi Organization protein 2 (TANGO2) deficiency has recently been identified as a rare metabolic disorder with a distinct clinical and biochemical phenotype of recurrent metabolic crises, hypoglycemia, lactic acidosis, rhabdomyolysis, arrhythmias, and encephalopathy with cognitive decline. We report nine subjects from seven independent families, and we studied muscle histology, respiratory chain enzyme activities in skeletal muscle and proteomic signature of fibroblasts. All nine subjects carried autosomal recessive TANGO2 mutations.

Generation of Microsecond Charge-Separated Excited States in Rhenium(I) Diimine Complexes: Driving Force Is the Dominant Factor in Controlling Lifetime.

A transition-metal-based donor-(linker)-acceptor system can produce long-lived charge transfer excited states using visible excitation wavelengths. The ground- and excited-state photophysical properties of a series of [ReCl(CO)(dppz-(linker)-TPA)] complexes, with varying donor and acceptor energies, have been systematically studied using spectroscopic techniques (both vibrational and electronic) supported by computational chemistry. The long-lived excited state is ILCT in nature for all complexes studied, characterized through transient absorption and emission, transient resonance Raman (TR), and time-resolved infrared (TRIR) spectroscopy and TDDFT calculations.

Spring constant and sensitivity calibration of FluidFM micropipette cantilevers for force spectroscopy measurements.

The fluidic force microscope (FluidFM) can be considered as the nanofluidic extension of the atomic force microscope (AFM). This novel instrument facilitates the experimental procedure and data acquisition of force spectroscopy (FS) and is also used for the determination of single-cell adhesion forces (SCFS) and elasticity. FluidFM uses special probes with an integrated nanochannel inside the cantilevers supported by parallel rows of pillars.

Monitoring the Formation and Reactivity of Organometallic Alkane and Fluoroalkane Complexes with Silanes and Xe Using Time-Resolved X-ray Absorption Fine Structure Spectroscopy.

Complexes with weakly coordinating ligands are often formed in chemical reactions and can play key roles in determining the reactivity, particularly in catalytic reactions. Using time-resolved X-ray absorption fine structure (XAFS) spectroscopy in combination with time-resolved IR (TRIR) spectroscopy and tungsten hexacarbonyl, W(CO), we are able to structurally characterize the formation of an organometallic alkane complex, determine the W-C distances, and monitor the reactivity with silane to form an organometallic silane complex. Experiments in perfluorosolvents doped with xenon afford initially the corresponding solvated complex, which is sufficiently reactive in the presence of Xe that we can then observe the coordination of Xe to the metal center, providing a unique insight into the metal-xenon bonding.

Efficacy of pulsed-xenon ultraviolet light for disinfection of high-touch surfaces in an Ecuadorian hospital.

Background: Hospital environment in patient care has been linked on healthcare-associated infections (HAI). No touch disinfection technologies that utilize pulsed xenon ultraviolet light has been recognized to prevent infection in contaminated environments. The purpose of this study was: 1) to evaluate the effectiveness of pulsed-xenon ultraviolet light (PX-UV) disinfection for the reduction of bacteria on environmental surfaces of Hospital General Enrique Garcés, and 2) to evaluate the in-vitro efficacy against multi-drug resistance microorganisms.

Identification of Cellular Pathogenicity Markers for SIL1 Mutations Linked to Marinesco-Sjögren Syndrome.

Recessive mutations in the gene cause Marinesco-Sjögren syndrome (MSS), a rare neuropediatric disorder. MSS-patients typically present with congenital cataracts, intellectual disability, cerebellar ataxia and progressive vacuolar myopathy. However, atypical clinical presentations associated with mutations have been described over the last years; compound heterozygosity of SIL1 missense mutations even resulted in a phenotype not fulfilling the clinical diagnostic criteria of MSS.