United States Department of Energy

About: United States Department of Energy is a government organization based out in Washington D.C., District of Columbia, United States. It is known for research contribution in the topics: Catalysis & Coal. The organization has 13656 authors who have published 14177 publications receiving 556962 citations. The organization is also known as: DOE & Department of Energy.

Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production

Abstract: Autosomal recessive mutations in proteasome subunit β 8 (PSMB8), which encodes the inducible proteasome subunit β5i, cause the immune-dysregulatory disease chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE), which is classified as a proteasome-associated autoinflammatory syndrome (PRAAS). Here, we identified 8 mutations in 4 proteasome genes, PSMA3 (encodes α7), PSMB4 (encodes β7), PSMB9 (encodes β1i), and proteasome maturation protein (POMP), that have not been previously associated with disease and 1 mutation in PSMB8 that has not been previously reported. One patient was compound heterozygous for PSMB4 mutations, 6 patients from 4 families were heterozygous for a missense mutation in 1 inducible proteasome subunit and a mutation in a constitutive proteasome subunit, and 1 patient was heterozygous for a POMP mutation, thus establishing a digenic and autosomal dominant inheritance pattern of PRAAS. Function evaluation revealed that these mutations variably affect transcription, protein expression, protein folding, proteasome assembly, and, ultimately, proteasome activity. Moreover, defects in proteasome formation and function were recapitulated by siRNA-mediated knockdown of the respective subunits in primary fibroblasts from healthy individuals. Patient-isolated hematopoietic and nonhematopoietic cells exhibited a strong IFN gene-expression signature, irrespective of genotype. Additionally, chemical proteasome inhibition or progressive depletion of proteasome subunit gene transcription with siRNA induced transcription of type I IFN genes in healthy control cells. Our results provide further insight into CANDLE genetics and link global proteasome dysfunction to increased type I IFN production.

Searching for a Better Thermal Battery

Abstract: Energy storage has mainly focused on electrochemical systems ( 1 ) However, more than 90% of the world's primary energy generation is consumed or wasted thermally Thermal energy storage has a broad and critical role to play in making energy use more sustainable for heating and cooling, solar energy harvesting, and other applications Thermal storage technologies are still based on solutions developed decades ago, such as molten salt, ice, and paraffin phase-change systems, whose performance and cost do not merit widerscale adoption Progress in materials science, chemistry, and engineering may lead to dramatic breakthroughs in thermal energy storage that could improve the efficiency with which we produce, distribute, and consume energy

Influence of harmonics on power distribution system protection

Abstract: The effects of nonsinusoidal voltages and currents on the performance of static underfrequency and overcurrent relays are experimentally studied. The tests were conducted in such a way that the frequency, amplitude and phase shift of individual harmonics could be adjusted in a controlled manner by using a waveform generator with a phase-locking circuit. The relationship between the harmonic currents and voltages was modeled in terms of the power system impedance within residential distribution systems. It was found that for harmonic voltage and current amplitudes (as they occur in distribution systems), underfrequency relays and the time delay operation of overcurrent relays show a marked deterioration in performance. The instantaneous operating characteristics of overcurrent relays, however, are hardly affected by the presence of harmonic currents. >

Rate of CO2 attack on hydrated Class H well cement under geologic sequestration conditions.

Abstract: Experiments were conducted to study the degradation of hardened cement paste due to exposure to CO2 and brine under geologic sequestration conditions (T = 50 °C and 30.3 MPa). The goal was to determine the rate of reaction of hydrated cement exposed to supercritical CO2 and to CO2-saturated brine to assess the potential impact of degradation in existing wells on CO2 storage integrity. Two different forms of chemical alteration were observed. The supercritical CO2 alteration of cement was similar in process to cement in contact with atmospheric CO2 (ordinary carbonation), while alteration of cement exposed to CO2-saturated brine was typical of acid attack on cement. Extrapolation of the hydrated cement alteration rate measured for 1 year indicates a penetration depth range of 1.00 ± 0.07 mm for the CO2-saturated brine and 1.68 ± 0.24 mm for the supercritical CO2 after 30 years. These penetration depths are consistent with observations of field samples from an enhanced oil recovery site after 30 years of ex...

Nucleation ahead of the advancing interface in directional solidification

Abstract: During directional solidification of an alloy, it is possible to nucleate the growing phase or a new phase at or ahead of the interface. This is critical in the phase selection, in the columnar to equiaxed transition under casting, welding or rapid solidification conditions and the formation of bands in peritectic systems. Following Hunt, an appropriate theoretical model is developed to determine the conditions under which nucleation can occur in the liquid close to a moving solid-liquid interface for both, low and high interface velocities. At high growth rates, non-equilibrium effects are shown to play an important role in predicting such transitions.