Cholesteryl ester storage disease. Report of a case.

Cholesteryl ester storage disease (CESD) is a rare disorder of familial incidence characterized by the accumulation of cholesteryl ester and triglycerides in the liver, intestine and bone marrow. Until now only 21 cases have been reported in the literature. We present a 9 months old girl presenting with increased abdominal girth. She had normal liver function tests and increased cholesterol and triglycerides serum levels. The liver biopsy showed many cholesterol cristals seen as needle shaped cristals under polarized light. This is the youngest patient being diagnosed clinically in the literature.

EFEITO DE SURFACTANTES NA ESTABILIDADE DE COMPOSTOS DE USO TERAPEUTICO

A critical revision of literature as regards to the drug stability in the presence of surfactants were realized. The functional groups envolved in the drug decomposition were used to the development of the discussion. The analysis indicated that the detergent effect can be used to control the rates and mechanisms of drug decomposition and to obtain specific information about the drug reactivity in the environment of pharmacological action.

Effects of HCl on the ultrasound catalyzed TEOS hydrolysis as determined by a calorimetric study

The acid catalyzed and ultrasound stimulated hydrolysis of solventless tetraethoxysilane-water mixtures was studied at 39°C as a function of HCl added to the mixtures (log[HCl]-1 ranged from 0.8 to 2.0), The reaction was carried out in a specially designed device, in which a steady state heat flow is maintained, while sonication is taking place, if no reaction is expected to occur. The exothermal hydrolysis reaction causes an increasing temperature (ΔTt) as a function of the reaction time, t. The isothermal hydrolysis rate constant, k, has been evaluated from the experimental ΔTt versus t data, after corrections for the increasing temperature effects, by using a method resulting from our theoretical modeling based on a dissolution and reaction mechanism. The hydrolysis rate constant fits closely a k α [H+] law as expected for this kind of hydrogen-ion catalyzed reaction.

Comparative time-course study of aminoacyl- and dipeptidyl-resin hydrolysis

The classic hydrolysis procedure for quantification of resin-bound aminoacyl and peptidyl groups with 12 N HCl: propionic acid was recvaluated by studying the influence of the nature of the resin and the resin-bound group. Their stability during acid hydrolysis was dependent on the C-terminal amino acid, and the order of acid stability was Phe > Val > Gly. Otherwise, the dipeptides Ala-Gly, Ala-Val, and Ala-Phe displayed enhanced rates of hydrolysis of the resin if compared with their parent aminoacyl groups. Amongthe resins assayed, the order of acid stability was: benzhydrylamine-resin > p-methylbenzhydrylamine-resin ≅4-(oxymethyl)-phenylacetamidomethyl-resin > chloromethyl-copolymer of styrene-1%-divinylbenzene. Important for peptide synthesis method, the findings demonstrate that longer hydrolysis times than previously recommended in the literature (1 h at 130°C and 15 min at 160°C for peptides attached to the chloromethyl-copolymer of styrene-1%-divinylbenzene) are necessary for the quantitative acid-catalyzed cleavage of some resin-bound groups. The observed broad range of hydrolysis time varied from less than 1 h to about 100 h.

Effect of micelles, microemulsions and other supramolecular aggregates on the hydrolysis of β-lactam antibiotics

A critical revision of the literature was made regarding the stability of β-lactam antibiotics in the presence of surfactants. The factors involved in the drug decomposition were analyzed in the development of the discussion. The analysis has indicated that some organized systems obtained from surfactants can be used to control rates and mechanisms of antibiotic decomposition. These organized systems can also be used to obtain specific information about the drug reactivity in a microenvironment similar to the site of pharmacological effect.

Formation of colloidal particles of hydrous iron oxide by forced hydrolysis

The mechanism of formation and growth of hydrous iron oxide (FeOOH) during the initial stages of forced hydrolyses of ferric chloride aqueous solution was studied by small angle X-ray scattering (SAXS). The effect of the hydrolysis temperature (60°C, 70°C and 80°C) and of the addition of urea on the formation of colloidal particles under isothermal conditions were investigated. Based on the experimental scattering functions in the Guinier range, we suggest the presence of elongated colloidal particles. The particle diameter and length, and their variation with time, were determined by fitting the form factor of prolate ellipsoids to the experimental scattering functions. We have assumed that our solutions are in a dilute state and that all colloidal particles are approximately of the same size. The colloidal particles have geometrical shapes similar to those of the subcrystals that build up the superstructure of β-FeOOH crystals, indicating that the formation of this hydrous iron oxide is governed by an aggregation process. Otherwise, the addition of urea hinders the growth and yields smaller particles, with a reduction in size greater than 50%. © 2000 Elsevier Science B.V. All rights reserved.

Optimization of neutral hydrolysis reaction of post-consumer PET for chemical recycling

The neutral hydrolysis reaction of post-consumer poly(ethylene terephthalate) in solid state was studied through the reaction of the polymer with water at the molar ratio 1:91 with autogenous pressure. Two sizes of post-consumer PET flakes and temperatures of 135 °C, 170°C and 205°C with pressures of 4.0 atm, 7.5 atm and 13.5 atm, respectively, were considered. With reaction time equal to 6h, the method reached 99% depolymerization at 205°C, 8.2% at 170 °C and 1.7% at 135°C. The reaction extension was measured by separating the terephthalic acid formed in the process and calculating by gravimetry how much material could still be reacted. Through the viscosimetry of diluted, solutions and the counting of carboxylic end groups in the remaining material from the gravimetric assay, it was possible to suggest that the reaction occurs randomly and in the whole volume of the polymeric particle and not solely on the surface. The terephthalic acid obtained and then purified was characterized by elemental analysis, magnetic nuclear resonance, size and panicle size distribution and spectrophotometry in the visible spectrum, and it was similar to the petrochemical equivalent, with purity recorded in carbon base equal to 99.9%.

Characterization of a tannase from Emericela nidulans immobilized on ionic and covalent supports for propyl gallate synthesis

The extracellular tannase from Emericela nidulans was immobilized on different ionic and covalent supports. The derivatives obtained using DEAE-Sepharose and Q-Sepharose were thermally stable from 60 to 75 °C, with a half life (t50) >24 h at 80 °C at pH 5. 0. The glyoxyl-agarose and amino-glyoxyl derivatives showed a thermal stability which was lower than that observed for ionic supports. However, when the stability to pH was considered, the derivatives obtained from covalent supports were more stable than those obtained from ionic supports. DEAE-Sepharose and Q-Sepharose derivatives as well as the free enzyme were stable in 30 and 50 % (v/v) 1-propanol. The CNBr-agarose derivative catalyzed complete tannic acid hydrolysis, whereas the Q-Sepharose derivative catalyzed the transesterification reaction to produce propyl gallate (88 % recovery), which is an important antioxidant. © 2012 Springer Science+Business Media Dordrecht.

Characterization of a glucose- and solvent-tolerant extracellular tannase from Aspergillus phoenicis

Tannases have attracted wider attention because of their biotechnological potential, especially enzymes from filamentous fungi and other microorganisms. However, the biodiversity of these microorganisms has been poorly explored, and few strains were identified for tannase production and characterization. This article describes the production, purification and characterization of a glucose- and solvent-tolerant extracellular tannase from Aspergillus phoenicis. High enzymatic levels were obtained in Khanna medium containing tannic acid up to 72 h at 30 °C under 100 rpm. The purified enzyme with 65% of carbohydrate content had an apparent native molecular mass of 218 kDa with subunits of 120 kDa and 93 kDa and was stable at 50 °C for 1 h. Optima of temperature and pH were 60 °C and 5.0-6.5, respectively. The enzyme was not affected significantly by most ions, detergents and organic solvents. While glucose did not affect the tannase activity, the addition of a high concentration of gallic acid did. The Km values were 1.7 mM (tannic acid), 14.3 mM (methyl-gallate) and 0.6 mM (propyl-gallate). The enzyme was able to catalyze the transesterification reaction to produce propyl-gallate. All biochemical properties suggest the biotechnological potential of the glucose- and solvent-tolerant tannase from A. phoenicis. © 2012 Elsevier B.V. All rights reserved.

Immobilization and stabilization of a bimolecular aggregate of the lipase from Pseudomonas fluorescens by multipoint covalent attachment

The soluble lipase from Pseudomonas fluorescens (PFL) forms bimolecular aggregates in which the hydrophobic active centers of the enzyme monomers are in close contact. This bimolecular aggregate could be immobilized by multipoint covalent linkages on glyoxyl supports at pH 8.5. The monomer of PFL obtained by incubation of the soluble enzyme in the presence of detergent (0.5% TRITON X-100) could not be immobilized under these conditions. The bimolecular aggregate has two amino terminal residues in the same plane. A further incubation of the immobilized derivative under more alkaline conditions (e.g., pH 10.5) allows a further multipoint attachment of lysine (Lys) residues located in the same plane as the amino terminal residues. Monomeric PFL was immobilized at pH 10.5 in the presence of 0.5% TRITON X-100. The properties of both PFL derivatives were compared. In general, the bimolecular derivatives were more active, more selective and more stable both in water and in organic solvents than the monomolecular ones. The bimolecular derivative showed twice the activity and a much higher selectivity (100 versus 20) for the hydrolysis of R,S-2-hydroxy-4-phenylbutyric acid ethyl ester (HPBEt) in aqueous media at pH 5.0 compared to the monomeric derivative. In experiments measuring thermal inactivation at 75 °C, the bimolecular derivative was 5-fold more stable than the monomeric derivative (and 50-fold more stable than a one-point covalently immobilized PFL derivative), and it had a half-life greater than 4 h. In organic solvents (cyclohexane and tert-amyl alcohol), the bimolecular derivative was much more stable and more active than the monomeric derivative in catalyzing the transesterification of olive oil with benzyl alcohol. © 2012 Elsevier Ltd. All rights reserved.

Solid-state hydrolysis of postconsumer polyethylene terephthalate after plasma treatment

Plasma treatments were applied on the surface of postconsumer polyethylene terephthalate (PET) bottles to increase their wettability and hasten the subsequent hydrolysis process. Sixty-four treatments were tested by varying plasma composition (oxygen and air), power (25-130 W), pressure (50-200 mTorr), and time (1 and 5 min). The best treatment was the one applied in air plasma at 130 W and 50 mTorr for 5 min, as it provided the lowest contact angle, 9.4°. Samples of PET before and after the optimized plasma condition were subjected to hydrolysis at 205°C. Although the treatment changed only a thin surface layer, its influence was evident up to relatively high conversion rates, as the treated samples presented more than 40% higher conversion rates than the untreated ones after 2 h of reaction. Infrared spectroscopy showed that the terephthalic acid obtained from 99% of depolymerization was similar to the commercial product used in PET synthesis. © 2012 Wiley Periodicals, Inc.

Characterization of esterase patterns in hepatopancreas of three species of Macrobrachium (Palaemonidae)

The genus Macrobrachium (Bate, 1868) belongs to the Palaemonidae family. These species are commonly found in lakes, floodplains and rivers in tropical and subtropical regions of South America. The Macrobrachium genus encompasses nearly 210 species of ecological and economic importance. In this study, three species of Macrobrachium (M acrobrachium jelskii, M acrobrachium amazonicum and M acrobrachium brasiliense) were studied in order to characterize the esterase patterns in the hepatopancreas, which were still unknown. Esterases are enzymes which catalyze the hydrolysis of esters. In the hepatopancreas, these enzymes play important roles in several metabolic processes involved in some functions of this organ, such as detoxification and digestion. Twelve esterase bands (EST1 to EST12) were detected in these species, and a comparison among them showed no qualitative differences in interspecific bands, or between males and females. Inhibitors were used to classify the esterase bands. The results indicated seven acetylesterases, two carboxylesterases, one arylesterase, and one cholinesterase. The EST11 band was not detected in these procedures because of its lower frequency. Statistical analyses showed no variability among the species, in either interspecific or intraspecific assays. These results support the hypothesis of a high evolutionary conservation of esterases in the hepatopancreas of these crustaceans. The data enabled us to assess the genetic structure of these species through the use of esterasic enzymes. It also contributes to our knowledge about the biology of these poorly studied species. Knowledge on the genetic structure of populations and species are essential when defining priorities for their management and conservation. © 2012 Elsevier Ltd.

Xylo-oligosaccharides from lignocellulosic materials: Chemical structure, health benefits and production by chemical and enzymatic hydrolysis

Currently, there is worldwide interest in the technological use of agro-industrial residues as a renewable source of food and biofuels. Lignocellulosic materials (LCMs) are a rich source of cellulose and hemicellulose. Hemicellulose is rich in xylan, a polysaccharide used to develop technology for producing alcohol, xylose, xylitol and xylo-oligosaccharides (XOSs). The XOSs are unusual oligosaccharides whose main constituent is xylose linked by β 1-4 bonds. The XOS applications described in this paper highlight that they are considered soluble dietary fibers that have prebiotic activity, favoring the improvement of bowel functions and immune function and having antimicrobial and other health benefits. These effects open a new perspective on potential applications for animal production and human consumption. The raw materials that are rich in hemicellulose include sugar cane bagasse, corncobs, rice husks, olive pits, barley straw, tobacco stalk, cotton stalk, sunflower stalk and wheat straw. The XOS-yielding treatments that have been studied include acid hydrolysis, alkaline hydrolysis, auto-hydrolysis and enzymatic hydrolysis, but the breaking of bonds present in these compounds is relatively difficult and costly, thus limiting the production of XOS. To obviate this limitation, a thorough evaluation of the most convenient methods and the opportunities for innovation in this area is needed. Another challenge is the screening and taxonomy of microorganisms that produce the xylanolytic complex and enzymes and reaction mechanisms involved. Among the standing out microorganisms involved in lignocellulose degradation are Trichoderma harzianum, Cellulosimicrobium cellulans, Penicillium janczewskii, Penicillium echinulatu, Trichoderma reesei and Aspergillus awamori. The enzyme complex predominantly comprises endoxylanase and enzymes that remove hemicellulose side groups such as the acetyl group. The complex has low β-xylosidase activities because β-xylosidase stimulates the production of xylose instead of XOS; xylose, in turn, inhibits the enzymes that produce XOS. The enzymatic conversion of xylan in XOS is the preferred route for the food industries because of problems associated with chemical technologies (e.g., acid hydrolysis) due to the release of toxic and undesired products, such as furfural. The improvement of the bioprocess for XOS production and its benefits for several applications are discussed in this study. © 2012 Elsevier Ltd.

Purification and Characterization of an Ethanol-Tolerant β-Glucosidase from Sporidiobolus pararoseus and Its Potential for Hydrolysis of Wine Aroma Precursors

An extracellular ethanol-tolerant β-glucosidase from Sporidiobolus pararoseus was purified to homogeneity and characterized, and its potential use for the enhancement of wine aroma was investigated. The crude enzymatic extract was purified in four steps (concentration, dialysis, ultrafiltration, and chromatography) with a yield of around 40 % for total activity. The purified enzyme (designated Sp-βgl-P) showed a specific activity of approximately 20.0 U/mg, an estimated molecular mass of 63 kDa after sodium dodecyl sulfate polyacrylamide gel electrophoresis, and isoelectric point of 5.0 by isoelectric focusing. Sp-βgl-P has optimal activity at pH 4.0 and at 55 °C. It was stable in a broad pH range at low temperatures and it was tolerant to ethanol and glucose, indicating suitable properties for winemaking. The hydrolysis of glycosidic terpenes was analyzed by adding Sp-βgl-P directly to the wines. The released terpene compounds were evaluated by gas chromatography/mass spectrometry. The enzymatic treatment significantly increased the amount of free terpenes, suggesting that this enzyme could potentially be applicable in wine aroma improvement. © 2013 Springer Science+Business Media New York.

Efeitos silibina sobre modelo pré-eclâmpsia induzida em ratas por tratamento com Nω-Nitro-L-arginina metil ester

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