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Sökning: WFRF:(Witzel Benjamin)

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1.
  • Dorst, Johannes, et al. (författare)
  • Metabolic alterations precede neurofilament changes in presymptomatic ALS gene carriers
  • 2023
  • Ingår i: EBioMedicine. - : Elsevier. - 2352-3964. ; 90
  • Tidskriftsartikel (refereegranskat)abstract
    • Background: The emergence of potentially effective new therapies for genetic forms of amyotrophic lateral sclerosis (ALS) necessitates the identification of biomarkers to facilitate early treatment, prior to the onset of motor symptoms. Here, we sought to investigate whether metabolic alterations are detectable in presymptomatic ALS gene mutation carriers, and whether such alterations precede neurofilament light chain (NfL) changes in serum.Methods: Between 02/2014 and 11/2021, we prospectively studied 60 presymptomatic ALS gene mutation carriers (40% male, age 48.7 ± 14.9; 28 C9orf72, 22 SOD1, 10 other) compared to 73 individuals from the same families (47% male, age 47.4 ± 12.9) without pathogenic mutations as controls. Bioimpedance analysis (BIA) and indirect calorimetry were performed, and Body Mass Index (BMI), Fat Mass (FM), Body Fat Percentage, Body Water (BW), Lean Body Mass (LBM), Extracellular Mass (ECM), Body Cell Mass (BCM), ECM/BCM ratio, Cells Percentage, Phase Angle, Resting Metabolic Rate (RMR), Metabolic Ratio (MR), and NfL were measured. Participants and evaluators were blinded regarding gene carrier status.Findings: Presymptomatic ALS gene carriers showed reduced LBM (p = 0.02), BCM (p = 0.004), Cells Percentage (p = 0.04), BW (p = 0.02), Phase Angle (p = 0.04), and increased ECM/BCM ratio (p = 0.04), consistently indicating a loss of metabolically active body cells. While in C9orf72 mutation carriers all tissue masses were reduced, only metabolically active tissue was affected in SOD1 mutation carriers. Unexpectedly, RMR (p = 0.009) and MR (p = 0.01) were lower in presymptomatic ALS gene carriers compared to non-carriers. NfL serum levels were similar in mutation carriers and non-carriers (p = 0.60).Interpretation: The observed metabolic phenomena might reflect reduced physical activity and/or preemptive, insufficient compensatory mechanisms to prepare for the later hypermetabolic state. As pre-symptomatic biomarkers we propose ECM/BCM ratio and Phase Angle for SOD1, and a 4-compartment affection in BIA for C9orf72 mutation carriers.
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2.
  • Nau, Patrick, et al. (författare)
  • Fiber-coupled phosphor thermometry for wall temperature measurements in a full-scale hydrogen gas turbine combustor
  • 2023
  • Ingår i: Measurement Science and Technology. - 0957-0233. ; 34:10
  • Tidskriftsartikel (refereegranskat)abstract
    • Online wall temperatures were measured with fiber-coupled phosphor thermometry in a full-scale gas turbine combustor. The combustor was operated with natural gas and up to 100 vol% hydrogen at engine-relevant conditions. Two phosphors were tested for this application, namely YAG:Dy and YAG:Tm;Li. Although YAG:Tm;Li seemed to be the most promising phosphor for this application, it turned out to be incompatible with the used setup due to a strong interfering signal generated by the laser in the used fiber setup. A strategy to compensate for interferences from flame emissions during natural gas operation was developed. With this strategy it was possible to obtain single-shot temperature measurements at 15 Hz and a precision of 2-7 K for a 1 s average.
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