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Glucokinase is essential for glucose-stimulated insulin release from the pancreatic beta-cell, serving as glucose sensor in humans. Inactivating or activating mutations of glucokinase lead to different forms of glucokinase disease, i.e. GCK-monogenic diabetes of youth, permanent neonatal diabetes (inactivating mutations), and congenital hyperinsulinism, respectively. Here we present a novel glucokinase gene (GCK)-activating mutation (p.E442K) found in an infant with neonatal hypoglycemia (1.5 mmol/liter) and in two other family members suffering from recurrent hypoglycemic episodes in their childhood and adult life. In contrast to the severe clinical presentation in the index case, functional studies showed only a slight activation of the protein (relative activity index of 3.3). We also report on functional studies of two inactivating mutations of the GCK (p.E440G and p.S441W), contiguous to the activating one, that lead to monogenic diabetes of youth. Interestingly, adult family members carrying the GCK pE440G mutation show an unusually heterogeneous and progressive diabetic phenotype, a feature not typical of GCK-monogenic diabetes of youth. In summary, we identified a novel activating GCK mutation that although being associated with severe neonatal hypoglycemia is characterized by the mildest activation of the glucokinase enzyme of all previously reported

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The Fraunhofer Institute for Environmental, Safety, and Energy Technology (Fraunhofer UMSICHT) at Sulzbach-Rosenberg, is developing a new method of intermediate pyrolysis, followed by reforming. The patented process, called Thermo-Catalytic-Reforming (TCR®), is the core technology in the To-Syn-Fuel project. The aim of the project is the production of advanced synthetic fuels from residual and waste materials (in particular sewage sludge), which are converted into the products bio-oil, as well as hydrogen, by means of the TCR®- plans. On the laboratory and pilot scale, the potential of this process for the generation of high-quality fuels according to the European standards EN 228 (for gasoline fuel) and EN 590 (for diesel fuel) has been already demonstrated, now the scale up of the plant is in the phase of commissioning. The aim of this work is to demonstrate the feasibility of TCR® gas cleaning from H2S and NH3 through the use of a scrubber. Several experiments have been carried out using FeCl2 as a scrubbing agent, from laboratory small scale to the real size scale plant, simulating the ideal gas concentration and operating condition of TCR500 plant. The best conditions for scrubbing H2S and NH3 have been investigated testing the stoichiometric reaction capacity of FeCl2 and the best environment inside the reactor. Moreover, new upgrades, such as sedimentation tank, filter, working bulk-absorption system, and a bypass, are tested to improve the scrubbing system in order to establish more efficient results and as well as have fewer issues related to lower byproduct formation, and decreasing of the maintenance time requested by the scrubber. These experiments reached 95% scrubbing efficiency and let a deep know-how for the control of the cleaning system and a good starting point for its integration in the plant start-up.

The Fraunhofer Institute for Environmental, Safety, and Energy Technology (Fraunhofer UMSICHT) at Sulzbach-Rosenberg, is developing a new method of intermediate pyrolysis, followed by reforming. The patented process, called Thermo-Catalytic-Reforming (TCR®), is the core technology in the To-Syn-Fuel project. The aim of the project is the production of advanced synthetic fuels from residual and waste materials (in particular sewage sludge), which are converted into the products bio-oil, as well as hydrogen, by means of the TCR®- plans. On the laboratory and pilot scale, the potential of this process for the generation of high-quality fuels according to the European standards EN 228 (for gasoline fuel) and EN 590 (for diesel fuel) has been already demonstrated, now the scale up of the plant is in the phase of commissioning. The aim of this work is to demonstrate the feasibility of TCR® gas cleaning from H2S and NH3 through the use of a scrubber. Several experiments have been carried out using FeCl2 as a scrubbing agent, from laboratory small scale to the real size scale plant, simulating the ideal gas concentration and operating condition of TCR500 plant. The best conditions for scrubbing H2S and NH3 have been investigated testing the stoichiometric reaction capacity of FeCl2 and the best environment inside the reactor. Moreover, new upgrades, such as sedimentation tank, filter, working bulk-absorption system, and a bypass, are tested to improve the scrubbing system in order to establish more efficient results and as well as have fewer issues related to lower byproduct formation, and decreasing of the maintenance time requested by the scrubber. These experiments reached 95% scrubbing efficiency and let a deep know-how for the control of the cleaning system and a good starting point for its integration in the plant start-up.