Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children

Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of OAS1, OAS2, or RNASEL in five unrelated children with MIS-C. The cytosolic double-stranded RNA (dsRNA)-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates (2-5A) that activate the single-stranded RNA-degrading ribonuclease L (RNase L). Monocytic cell lines and primary myeloid cells with OAS1, OAS2, or RNase L deficiencies produce excessive amounts of inflammatory cytokines upon dsRNA or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) stimulation. Exogenous 2-5A suppresses cytokine production in OAS1-deficient but not RNase L-deficient cells. Cytokine production in RNase L-deficient cells is impaired by MDA5 or RIG-I deficiency and abolished by mitochondrial antiviral-signaling protein (MAVS) deficiency. Recessive OAS-RNase L deficiencies in these patients unleash the production of SARS-CoV-2-triggered, MAVS-mediated inflammatory cytokines by mononuclear phagocytes, thereby underlying MIS-C

The Structural and Electrical Study of Lu-Doped YBCO System

WOS: 000323906600099We have prepared a series of bulk superconducting samples with the nominal composition of Y1-xLuxBa2 Cu3Oy (where x = 0.0, 0.05, 0.1, 0.2 and 0.3) by the conventional solid-state reaction method. The samples were characterized structurally by means of X-ray diffraction, scanning electron microscopy and energy dispersive X-ray spectrometry. The electrical transport properties of the samples were analyzed in the temperature range between 20-140 K under magnetic fields up to 2 T. The superconducting transition temperature, T-c, and activation energy, U-0, were found to decrease with Lu-doping and with increase in applied magnetic field

On the ionic conductivity of polymer electrolytes in terms of hole fraction

A theoretical model to interpret the conductivity of ions through polymer electrolytes is established in terms of the temperature and pressure-dependent hole fraction computed from Simha-Somcynsky hole theory. The model successfully linearizes the logarithm of PPG and PEG conductivity data with NaCF3SO3 in a 20:1 ratio for a broad range of temperature and pressure. The conductivity parameter and transmission coefficient with an additive hole fraction constant are discussed and compared for both species. The derivative of the logarithm of conductivity with respect to the hole fraction decreases inversely with the hole fraction and saturates at about 0.08 and 0.12 hole fractions for PPG and PEG, respectively. (C) 2008 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 46: 2249-2254, 200