A novel, dominant disease mechanism of distal renal tubular acidosis with specific variants in ATP6V1B1.

Background And Hypothesis: ATP6V1B1 encodes a subunit of the vacuolar H+-ATPase and pathogenic variants are associated with autosomal recessive distal renal tubular acidosis (dRTA) with deafness. Heterozygous variants predicted to affect a specific amino acid, Arg394, have been recurrently reported in dRTA but their significance has been unclear. We hypothesised that these variants are associated with a dominant disease mechanism.

Prognostic value of the 1-min sit-to-stand test to predict post-operative complications in patients with lung cancer elected for lung resection.

https://bit.ly/3T7pnS9.

Assessing the impact of PV panel climate-based degradation rates on inverter reliability in grid-connected solar energy systems.

This paper provides an evaluation of a 4-kW grid-connected full-bridge PV inverter under three different scenarios to assess its reliability with a fixed PV degradation rate, with a climate-based degradation rate, and without considering PV degradation. The climate-based degradation rates are estimated using a physics-based model that considers the different parameters influencing the PV reliability. Three different locations representing three different climate zones (hot and dry, hot and humid, and moderate climates) have been chosen in this study.

Physical status, symptoms and health-related quality of life during a severe exacerbation of COPD: Recovery and discriminative capacity for future events.

Objective: Severe acute exacerbations of chronic obstructive pulmonary disease (AECOPD) can have a negative impact on functional capacity, symptoms and health-related quality of life (HRQOL). This study aimed to i) investigate the recovery of muscle strength, functional capacity, symptoms, and HRQOL in patients after a severe AECOPD; ii) compare with matched patients with stable COPD (SCOPD); and iii) assess whether these assessments at hospital discharge could discriminate patients' risk for future events.

Methods: This observational study assessed patients with AECOPD during hospital discharge (T1) and one month after discharge (T2).

Photo-Oxidizing Ruthenium(II) Complexes with Enhanced Visible-Light Absorption and G-quadruplex DNA Binding Abilities.

Photosensitizers that gather high photo-oxidizing power and strong visible-light absorption are of great interest in the development of new photo-chemotherapeutics. Indeed, such compounds constitute attractive candidates for the design of type I photosensitizers that are not dependent on the presence of oxygen. In this paper, we report on the synthesis and studies of new ruthenium(II) complexes that display strong visible-light absorption and can oxidize guanine residues under visible-light irradiation, as evidenced by nanosecond transient absorption spectroscopy.