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Beta-propeller Protein Associated Neurodegeneration (BPAN) is a devastating neurodevelopmental and neurodegenerative disease linked to variants inWDR45. Currently, there is no cure or disease altering treatment for this disease. This is, in part, due to a lack of insight into early phenotypes of BPAN progression andWDR45’s role in establishing and maintaining neurological function. Here we generated and characterized a mouse model bearing a c52C > T BPAN patient variant inWdr45.We show this mutation ablatesWDR45protein expression and alters autophagy in the brain. Behavioral analysis of these mice revealed characteristic signs of BPAN including cognitive impairment, hyperactivity, and motor decline. We show these behaviors coincide with widespread glial activation and early development of axonal spheroids in multiple neuron subclasses throughout the brain. Several lines of evidence suggest these spheroids arise from axon terminals. Transcriptomic analysis uncovered multiple disrupted pathways in the cortex including genes associated with synapses, neurites, endosomes, endoplasmic reticulum, and ferroptosis. This is supported by accumulation of the iron regulating transferrin receptor 1 (TFRC) and the endoplasmic reticulum resident calreticulin (CALR) in the cortex as these animals age. CALR forms spheroid structures similar to the axonal spheroids seen in these animals. Taken together, our data suggest that WDR45 is necessary for healthy brain function and maintenance of axon terminals. This model opens the door to therapeutics targeting BPAN and further exploration of the role of WDR45 in neuronal function. 

Abstract Aqueous‐phase uptake and processing of water‐soluble organic compounds can promote secondary organic aerosol (SOA) production. We evaluated the contributions of aqueous‐phase chemistry to summertime urban SOA at two sites in New York City. The relative role of aqueous‐phase processing varied with chemical and environmental conditions, with evident daytime SOA enhancements (e.g., >1 μg/m³) during periods with relative humidities (RH) exceeding 65% and often higher temperatures. Oxygenated organic aerosol (OOA) production was also sensitive to secondary inorganic aerosols, in part through their influence on aerosol liquid water. On average, high‐RH periods exhibited a 69% increase in less‐oxidized OOA production in Queens, NY. These enhancements coincided with southerly backward trajectories and greater inorganic aerosol concentrations, yet showed substantial intra‐city variability between Queens and Manhattan. The observed aqueous‐phase SOA production, even with historically low sulfate and nitrate aerosol loadings, highlights both opportunities and challenges for continued reductions in summertime PM_2.5in urban communities. 

Context.On 13 March 2023, when the Parker Solar Probe spacecraft (S/C) was situated on the far side of the Sun as seen from Earth, a large solar eruption took place, which created a strong solar energetic particle (SEP) event observed by multiple S/C all around the Sun. The energetic event was observed at six well-separated locations in the heliosphere, provided by the Parker Solar Probe, Solar Orbiter, BepiColombo, STEREO A, near-Earth S/C, and MAVEN at Mars. Clear signatures of an in situ shock crossing and a related energetic storm particle (ESP) event were observed at all inner-heliospheric S/C, suggesting that the interplanetary coronal mass ejection (CME)-driven shock extended all around the Sun. However, the solar event was accompanied by a series of pre-event CMEs. Aims.We aim to characterize this extreme widespread SEP event and to provide an explanation for the unusual observation of a circumsolar interplanetary shock and a corresponding circumsolar ESP event. Methods.We analyzed data from seven space missions, namely Parker Solar Probe, Solar Orbiter, BepiColombo, STEREO A, SOHO, Wind, and MAVEN, to characterize the solar eruption at the Sun, the energetic particle event, and the interplanetary context at each observer location as well as the magnetic connectivity of each observer to the Sun. We then employed magnetohydrodynamic simulations of the solar wind in which we injected various CMEs that were launched before as well as contemporaneously with the solar eruption under study. In particular, we tested two different scenarios that could have produced the observed global ESP event: (1) a single circumsolar blast-wave-like shock launched by the associated solar eruption, and (2) the combination of multiple CMEs driving shocks into different directions. Results.By comparing the simulations of the two scenarios with observations, we find that both settings are able to explain the observations. However, the blast-wave scenario performs slightly better in terms of the predicted shock arrival times at the various observers. Conclusions.Our work demonstrates that a circumsolar ESP event, driven by a single solar eruption into the inner heliosphere, is a realistic scenario. 

Understanding the microscopic origin of the superior electromechanical response in relaxor ferroelectrics requires knowledge not only of the atomic-scale formation of polar nanodomains (PNDs) but also the rules governing the arrangements and stimulated response of PNDs over longer distances. Using x-ray coherent nanodiffraction, we show the staggered self-assembly of PNDs into unidirectional mesostructures that we refer to as polar laminates in the relaxor ferroelectric 0.68PbMg_1/3Nb_2/3O₃-0.32PbTiO₃(PMN-0.32PT). We reveal the highly heterogeneous electric-field–driven responses of intra- and interlaminate PNDs and establish their correlation with the local strain and the nature of the PND walls. Our observations highlight the critical role of hierarchical lattice organizations on macroscopic material properties and provide guiding principles for the understanding and design of relaxors and a wide range of quantum and functional materials. 

We have investigated the influence of non-stoichiometry and local atomic environments on carrier transport in GaAs(N)Bi alloy films using local-electrode atom probe tomography (LEAP) in conjunction with time-resolved terahertz photoconductivity measurements. The local concentrations of N, Bi, and excess As, as well as Bi pair correlations, are quantified using LEAP. Using time-resolved THz photoconductivity measurements, we show that carrier transport is primarily limited by excess As, with the highest carrier mobilities for layers with yBi > 0.035.