Publications by authors named "F Porter"

This paper presents progress made toward the overarching goal to adapt single-photon-counting microcalorimeters to magnetic fusion energy research and demonstrate the value of such measurements for fusion. Microcalorimeter spectrometers combine the best characteristics of x-ray instrumentation currently available on fusion devices: high spectral resolution similar to an x-ray crystal spectrometer and broad spectral coverage sufficient to measure impurity species from Be to W. As a proof-of-principle experiment, a NASA-built x-ray microcalorimeter spectrometer has been installed on the Madison Symmetric Torus (MST) at the Wisconsin Plasma Physics Laboratory.

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Niemann-Pick disease, type C1 (NPC1) is an ultra rare, autosomal recessive disorder characterized by impaired intracellular cholesterol trafficking. This study assessed neuron-specific enolase (NSE) as a biomarker for disease status and treatment response in individuals with NPC1. We also evaluated the concordance between serum and cerebrospinal fluid (CSF) NSE measurements.

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Article Synopsis
  • The Lunar Environment heliospheric X-ray Imager (LEXI) is designed to capture x-ray images from solar wind interactions in Earth's magnetosheath, helping researchers understand energy transfer into Earth's magnetosphere.
  • As part of a lunar lander mission landing in Mare Crisium, LEXI features a unique array of 48 neodymium magnets to protect against interference from charged particles while minimizing stray magnetic fields.
  • A Runge-Kutta-based simulation model shows that LEXI's deflector array and other particle suppression methods effectively limit proton and electron contamination, ensuring the success of its imaging capabilities and offering insights for similar instruments.
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Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by pathological variants in DHCR7, resulting in a deficiency in the enzyme 7-dehydrocholesterol reductase. This results in elevated levels of cholesterol precursors and typically low cholesterol levels, leading to a range of physical and cognitive challenges. Mortality rates in infants with severe SLOS are high, due to congenital malformations.

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Smith-Lemli-Opitz syndrome (SLOS) is a rare, multiple malformation/intellectual disability disorder caused by pathogenic variants of DHCR7. DHCR7 catalyzes the reduction of 7-dehydrocholesterol (7DHC) to cholesterol in the final step of cholesterol biosynthesis. This results in accumulation of 7DHC and a cholesterol deficiency.

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