Mucopolysaccharidoses: Cellular Consequences of Glycosaminoglycans Accumulation and Potential Targets

Mucopolysaccharidoses (MPSs) constitute a heterogeneous group of lysosomal storage disorders characterized by the lysosomal accumulation of glycosaminoglycans (GAGs). Although lysosomal dysfunction is mainly affected, several cellular organelles such as mitochondria, endoplasmic reticulum, Golgi apparatus, and their related process are also impaired, leading to the activation of pathophysiological cascades. While supplying missing enzymes is the mainstream for the treatment of MPS, including enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), or gene therapy (GT), the use of modulators available to restore affected organelles for recovering cell homeostasis may be a simultaneous approach. This review summarizes the current knowledge about the cellular consequences of the lysosomal GAGs accumulation and discusses the use of potential modulators that can reestablish normal cell function beyond ERT-, HSCT-, or GT-based alternatives.     

Microscopic and Spectroscopic Evaluation of Inactivation of <Emphasis Type="Italic">Staphylococcus aureus</Emphasis> by Pulsed UV Light and Infrared Heating

Pulsed UV light and infrared heat-treated Staphylococcus aureus cells were analyzed using transmission electron microscopy to identify the cell damage due to the treatment process. A 5-s treatment with pulsed UV light resulted in complete inactivation of S. aureus even after enrichment. The temperature increase during the pulsed UV light treatment was insignificant, which suggested a nonthermal treatment. S. aureus was also infrared heat treated using an infrared heating system with six infrared lamps. Five milliliters of S. aureus cells in phosphate buffer was treated at 700°C lamp temperature for 20 min. The microscopic observation clearly indicated that there was cell wall damage, cytoplasmic membrane shrinkage, cellular content leakage, and mesosome disintegration after both pulsed UV light and infrared treatments. Fourier transform infrared microspectrometry was successfully used to classify the pulsed UV light and infrared heat-treated S. aureus by discriminant analysis.     

Modeling of pullulan fermentation by using a color variant strain of Aureobasidium pullulans

Pullulan, which is comprised of glucose units, is a simple linear polysaccharide produced by Aureobasidium pullulans. Pullulan has long been used in various applications such as blood plasma substitutes, food additives, adhesive additives, flocculants, and even environmental pollution control agents. Mathematical models of biomass, pullulan, and sucrose profiles during fermentation not only provide information about the kinetic-metabolic nature of pullulan, but also facilitate the control and optimization of pullulan production. In this study, several models were modified and tested in order to describe biomass, pullulan, and sucrose profiles during batch fermentation using a color variant strain of A. pullulans. The results demonstrated that the modified Gompertz model can serve as a universal equation to fit biomass production, pullulan production, and sucrose consumption. Furthermore, validation of this modified Gompertz model indicated that biomass (slope = 1.00, R² = 0.991), pullulan (slope = 1.10, R² = 0.991), and sucrose (slope = 0.96, R² = 0.991) were all predicted accurately.     

A Survey of Barley,Malt and Beer Contamination with Ochratoxin A in Turkey

The presence of ochratoxin A (OTA) was investigated in barley, malt and beer samples using an ELISA method (RIDASCREEN). The lower detection limit of this OTA test was 0.08 μg/L for beer and 0.4 μg/kg for barley and malt. In 26 out of 29 barley samples the OTA content was between 0.53–12 μg/kg. OTA was between 0.5–6.6 μg/kg in 23 out of 24 malt samples. Only one malt sample had no detectable OTA using RIDASCREEN. The OTA content was between 0.1–8.10 μg/L in 42 out of 150 beer samples (28%) and in 108 beer samples OTA was not found at detectable levels (72%). Only one beer sample contained more than 5 μg/L OTA.     

Impact of H.I. Schlesinger's discoveries upon the course of modern chemistry on B−(N−)H hydrogen carriers

Representatives of B?(N?)H hydrogen carriers are alkali borohydrides (e.g. LiBH₄ and NaBH₄) and amine boranes (e.g. NH₃BH₃ and NaNH₂BH₃). These are old compounds; they were discovered in the first half of the 20th century. One of the main contributors to their development is Prof. Hermann I. Schlesinger (1882–1960). In the recent years, there has been new interest in these old compounds as novel chemical H storage materials. Sodium borohydride NaBH₄ is a typical example. It was discovered by Schlesinger and collaborators in the 1950s. At that time it was found to be an attractive H₂ generator; owing to this property it reemerged in the early 2000s for on-board H₂ generation. Today, Schlesinger is commonly considered as the pioneer of NaBH₄ as hydrogen carrier but the impact of Schlesinger and collaborators' discoveries upon the course of modern chemistry on B?(N?)H hydrogen carriers is far more extensive … This is highlighted herein.     

Very fast H2 production from the methanolysis of NaBH4 by metal‐free poly(ethylene imine) microgel catalysts

The polyethyleneimine (PEI) microgels prepared via microemulsion polymerization are protonated by hydrochloric acid treatment (p‐PEI) and quaternized (q‐PEI) via modification reaction with methyl iodide and with bromo alkanes of different alkyl chain lengths such as 1‐bromoethane, 1‐bromobutane, 1‐bromohexane, and 1‐bromooctane. The bare p‐PEI and q‐PEI microgels are used as catalysts directly without any metal nanoparticles for the methanolysis reaction of sodium borohydride (NaBH₄). Various parameters such as the protonation/quaternization reaction on PEI microgels, the amount of catalyst, the amount of NaBH₄, and temperature are investigated for their effects on the hydrogen (H₂) production rate. The reaction of self‐methanolysis of NaBH₄ finishes in about 32.5 min, whereas the bare PEI microgel as catalyst finishes the methanolysis of NaBH₄ in 22 min. Surprisingly, it is found that when the protonated PEI microgels are used as catalyst, the same methanolysis of NaBH₄ is finished in 1.5 min. The highest H₂ generation rate value is observed for protonated PEI microgels (10 mg) with 8013 mL of H₂/(g of catalyst.min) for the methanolysis of NaBH₄. Moreover, activation parameters are also calculated with activation energy value of 23.7 kJ/mol, enthalpy 20.9 kJ/mol, and entropy ?158 J/K.mol. Copyright © 2016 John Wiley & Sons, Ltd.     

Surface‐modified carbon black derived from used car tires as alternative,reusable, and regenerable catalysts for H2 release studies from sodium borohydride methanolysis

Carbon black (CB) obtained from used car tire rubbers were treated with concentrated sulfuric and nitric acids. The oxidized CB (CB‐COO‐Na⁺) is subsequently modified with epichlorohydrin (ECH) and amines including polyethylene imine (PEI). These modified CBs such as CB‐PEI are used as metal‐free catalysts in methanolysis of sodium borohydride (NaBH₄) to produce hydrogen. The hydrogen generation rate (HGR) of 3089 ± 44.69 mL.min^‐1.g^‐1 is accomplished at room temperature with CB‐PEI‐hydrochloric acid (HCl) catalyst. The resulting activation energy of 34.7 kJ/mol for the temperature range of ?20°C to +30°C compares favorably to most of alternative catalysts reported in literature while reaction catalyzing capabilities of CB‐PEI‐HCl particles extend to the subzero temperature range (?20°C‐0°C). The reuse and regeneration studies conducted for the CB‐PEI‐HCl catalyst showed that these catalysts do provide complete conversion at every use up to five consecutive runs and retain 50 ± 2.5% of the original hydrogen generation rate at the fifth consecutive reuse. The CBs‐based catalysts are fully regenerated with HCl treatment.     

Bimetallic RuCo and RuCu catalysts supported on γ-Al2O3. A comparative study of their activity in hydrolysis of ammonia-borane

Bimetallic-based RuCo and RuCu catalysts, supported on γ-Al₂O₃ (1.5 wt% Ru as theoretical value), were synthesized by polyol method. Ru, Co, and Cu acetylacetonates were used as precursors and ethylene glycol as reducing agent. The as-synthesized catalysts were characterized by SEM, TEM, XRD and XPS, and tested in ammonia-borane (NH₃BH₃) hydrolytic dehydrogenation at variable amount of catalyst (10-30 wt%), concentration of NH₃BH₃ (1.0-0.65 M), and temperatures (50-65 °C). The reactions were monitored by volumetric (inverted burette) and spectroscopic methods (¹¹B and ¹¹B{¹H} NMR). It was found that the best bimetallic catalysts are those having a molar ratio Ru:Co and Ru:Cu of 1:1 such as RuCo > RuCu ∼ Ru. They, i.e. RuCo and RuCu, consist of nanosized spherical particles of Ru⁰Co(OH)₂ and Ru⁰Cu⁰, respectively. Kinetic investigation highlights similar rate laws with activation energies of 47 kJ mol⁻¹ and 52 kJ mol⁻¹, respectively, and, for both, reaction orders of 1 versus both the NH₃BH₃ and the catalytic free sites concentrations. ¹¹B and ¹¹B{¹H} NMR investigation confirmed (i) a more effective NH₃BH₃ hydrolytic dehydrogenation in the presence of RuCo catalyst even though a loss of activity after the first run was observed for both catalysts, and (ii) a rapid NH₃BH₃ hydrolysis with initial formation of B(OH)₄⁻, which besides favors equilibriums of formation of polyborates. These results are reported and the reaction mechanism discussed herein.     

Ammonia borane thermolytic decomposition in the presence of metal (II) chlorides

In the present work, we studied the effect of metal chlorides, MCl₂, on the thermal decomposition of ammonia borane NH₃BH₃ (AB). Some metals from row n = 4 of the periodic table were chosen and used as MCl₂: namely, FeCl₂, CoCl₂, NiCl₂, CuCl₂, and ZnCl₂. In addition, three metals from column VIII of the periodic table were considered: NiCl₂, PdCl₂ and PtCl₂. The AB decomposition was followed by TGA and DSC; the decomposition gases analyzed by μGC/MSD coupling, and the solid by-products identified by XRD, IR and XPS. We observed that the presence of CuCl₂ in AB is beneficial, making the decomposition occur in much milder conditions than for pristine AB; for example, the dehydrogenation of CuCl₂-doped AB started at 25 °C, with the sample losing about 14 wt% at 85 °C. However, MCl₂ does not hinder the evolution of the undesired borazine; it only contributes to a decrease in its content compared to pristine AB. To rationalize the better performance of CuCl₂, we propose that Cu offers an optimal doping activity with intermediate binding energies for the intermediates: i.e. with H not too strongly bonded but optimally bonded to the N of AB. The germ Cu?NH₂–BH₂, then formed, acts as a Lewis acid through B and has an optimized reactivity towards a new AB molecule (head-to-tail dehydrocoupling). This is discussed herein.