Publications by authors named "A Kochanski"

Despite the fact that there are published case reports and model work providing evidence of inflammation in Charcot-Marie-Tooth disorders (CMTs), in clinical practice, CMT and inflammatory neuropathies are always classified as two separate groups of disorders. This sharp separation of chronic neuropathies into two groups has serious clinical implications. As a consequence, the patients harboring CMT mutations are practically excluded from pharmacological anti-inflammatory treatments.

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Article Synopsis
  • IGHMBP2 pathogenic variants are linked to two severe neurodegenerative diseases: the fatal spinal muscular atrophy with respiratory distress type 1 (SMARD1) and the milder Charcot-Marie-Tooth type 2S (CMT2S) neuropathy.
  • Despite over two decades of research, the specific mechanisms by which IGHMBP2 mutations cause these diseases remain unclear, although its role as an RNA/DNA helicase has been identified.
  • The review discusses the clinical characteristics of IGHMBP2-related diseases, available model systems for research, the protein's structure, and potential causes of neurodegeneration associated with these conditions.
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Wildland fire is a major global driver in the exchange of aerosols between terrestrial environments and the atmosphere. This exchange is commonly quantified using emission factors or the mass of a pollutant emitted per mass of fuel burned. However, emission factors for microbes aerosolized by fire have yet to be determined.

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California has experienced enhanced extreme wildfire behaviour in recent years, leading to substantial loss of life and property. Some portion of the change in wildfire behaviour is attributable to anthropogenic climate warming, but formally quantifying this contribution is difficult because of numerous confounding factors and because wildfires are below the grid scale of global climate models. Here we use machine learning to quantify empirical relationships between temperature (as well as the influence of temperature on aridity) and the risk of extreme daily wildfire growth (>10,000 acres) in California and find that the influence of temperature on the risk is primarily mediated through its influence on fuel moisture.

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