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3-Hydroxy-3-methylglutaryl-coenzyme A lyase (HMGCL, HMGCL) deficiency is a rare inborn error of ketogenesis. Even if the ketogenic enzyme is fully disrupted, an elevated signal for the ketone body acetoacetic acid is a frequent observation in the analysis of urinary organic acids, at least if derivatization is performed by methylation. We provide an explanation for this phenomenon and trace it back to degradation of the derivatized 3-hydroxy-3-methylglutaric acid and high temperature of the injector of the gas chromatograph.
Amino acids perform multiple essential physiological roles in humans, and accordingly, their importance to health has been the subject of extensive attention. In this special issue of the Journal of Nutrition and Metabolism, we focus on the various inborn errors of amino acid metabolism, their diagnostic challenges, new treatment approaches, and recent advances in patient monitoring as well as clinical outcomes.
Background: Local injection of autologous conditioned serum (ACS) is a well-known therapy for inflammatory diseases (IDs). While patients’ blood is incubated to generate ACS (with subsequent centrifugation), immune cells produce high amounts of growth factors and cytokines. This include, amongst others, interleukin-1 receptor antagonist (IL-1ra), interleukins 6 and 10, tumour necrosis factor alpha (TNF-α) and transforming growth factor beta 1 (TGF-β1). The aim of this study was to analyse exosomes release into ACS as well as their cytokine cargo.
Major progress occurred in understanding inborn errors of ketone body transport and metabolism between the International Congresses on Inborn Errors of Metabolism in Barcelona (2013) and Rio de Janeiro (2017). These conditions impair either ketogenesis (presenting as episodes of hypoketotic hypoglycemia) or ketolysis (presenting as ketoacidotic episodes); for both groups, immediate intravenous glucose administration is the most critical and (mHGGCS, HMGCS2) effective treatment measure.
Influence of design of extrusion blow molding (EBM) in terms of extrusion direction set-up and draw ratio as well as process conditions (mold temperature) on storage modulus of high density polyethylene EBM containers was analyzed with dynamic mechanical analysis. All three parameters - mold temperature, flow direction and draw ratio - are statistically significant and lead to relative and absolute evaluation of storage modulus. Furthermore, flow induced changes in crystallinity was analyzed by differential scanning calorimetry. Obtained data on deformation properties can be employed for more sophisticated finite element simulations with the aim to reach more sustainable extrusion blow molding production.
Gegenstand dieser Arbeit sind Untersuchungen zur Detektion von verpackten Gefahrstoffen wie beispielsweise Explosivstoffen. Hierzu wird in einem ersten Schritt die Verpackung mittels Laserbohrens durchdrungen, um anschließend den nun freiliegenden Gefahrstoff nachweisen zu können. Dies geschieht einerseits durch eine lasergestützte Probenahme und anschließende Detektion mit gängigen chemisch-analytischen Verfahren sowie direkt bei der Wechselwirkung zwischen Laser und Gefahrstoff mittels Ramanspektroskopie. Zudem werden schnelle in situ-Techniken im Hinblick auf ihre Eignung zur Überwachung des Laserbohrprozesses untersucht. Hier werden kostengünstige und kompakte Sensortechniken (Messung der Prozessgase durch Halbleitergassensoren, Messung des Luftschalls mittels Kondensatormikrofon) mit aufwendigeren und komplexeren spektroskopischen Verfahren (Plasma- und Ramanspektroskopie) bewertend verglichen. Anhand ausgewählter Modellsysteme in verkleinertem Maßstab werden die unterschiedlichen Verfahren unter Verwendung gängiger Verpackungs- und Hüllenmaterialien sowie anhand ausgewählter Explosivstoffe charakterisiert. Für das Laserverfahren kommen gepulste Nd:YAG Laser mit unterschiedlichen Emissionswellenlängen zum Einsatz.