Molecular and cellular pathogenesis of autosomal recessive polycystic kidney disease

Autosomal recessive polycystic kidney disease (ARPKD) is an inherited disease characterized by a malformation complex which includes cystically dilated tubules in the kidneys and ductal plate malformation in the liver. The disorder is observed primarily in infancy and childhood, being responsible for significant pediatric morbidity and mortality. All typical forms of ARPKD are caused by mutations in a single gene, PKHD1 (polycystic kidney and hepatic disease 1). This gene has a minimum of 86 exons, assembled into multiple differentially spliced transcripts and has its highest level of expression in kidney, pancreas and liver. Mutational analyses revealed that all patients with both mutations associated with truncation of the longest open reading frame-encoded protein displayed the severe phenotype. This product, polyductin, is a 4,074-amino acid protein expressed in the cytoplasm, plasma membrane and primary apical cilia, a structure that has been implicated in the pathogenesis of different polycystic kidney diseases. In fact, cholangiocytes isolated from an ARPKD rat model develop shorter and dysmorphic cilia, suggesting polyductin to be important for normal ciliary morphology. Polyductin seems also to participate in tubule morphogenesis and cell mitotic orientation along the tubular axis. The recent advances in the understanding of in vitro and animal models of polycystic kidney diseases have shed light on the molecular and cellular mechanisms of cyst formation and progression, allowing the initiation of therapeutic strategy designing and promising perspectives for ARPKD patients. It is notable that vasopressin V2 receptor antagonists can inhibit/halt the renal cystic disease progression in an orthologous rat model of human ARPKD.

Rheological behavior of corn and soy mix as feed ingredients

Foods behave as non-Newtonian fluids, but little is known about how corn and soybean mix behave under viscometric flow. In order to characterize the rheological behavior of animal feed under viscometric flow, a 70:30 (mass:mass) mixture of ground corn and soybean grains was submitted to a capillary rheometer at 3 different temperatures (80, 120, and 160 °C), different moisture levels (26.5 ± 0.08; 30.4 ± 0.31, and 33.4 ± 0.05%), and 4 shear rates (30.4; 72.9; 304.3, and 728.6/second). Different strain rates and die dimensions were used to obtain the target shear rates. The resulting data were fitted to Power Law, Casson, and Bingham models. Based on experimental data, water content, mass temperature, and the effects of shear rate on the apparent shear viscosity of corn-soy mix were fitted to a single expression (p < 0.001, R² = 0.93): η = 18,769.7 (y)-0.86 e (-9.34 U + 935 T), where y is shear rate, U is sample moisture, and T is sample temperature in Kelvin scale. As expected, such mixture presented a pseudoplastic (shear-thinning) behavior.

Chemical pattern of brazilian apples: a chemometric approach based on the Fuji and Gala varieties

The chemical composition of apple juices may be used to discriminate between the varieties for consumption and those for raw material. Fuji and Gala have a chemical pattern that can be used for this classification. Multivariate methods correlate independent continuous chemical descriptors with the categorical apple variety. Three main descriptors of apple juice were selected: malic acid, total reducing sugar and total phenolic compounds. A chemometric approach, employing PCA and SIMCA, was used to classify apple juice samples. PCA was performed with 24 juices from Fuji and Gala, and SIMCA, with 15 juices. The exploratory and predictive models recognized 88% and 64%, respectively, as belonging to a mixed domain. The apple juice from commercial fruits shows a pattern related to cv. Fuji and Gala with boundaries from 0.18 to 0.389 g.100 mL-1 (malic acid), from 8.65 to 15.18 g.100 mL-1 (total reducing sugar) and from 100 to 400 mg.L-1 (total phenolic compounds), but such boundaries were slightly shorter in the remaining set of commercial apple juices, specifically from 0.16 to 0.36 g.100 mL-1, from 9.25 to 15.5 g.100 mL-1 and from 180 to 606 mg.L-1 for acidity, reducing sugar and phenolic compounds, respectively, representing the acid, sweet and bitter tastes.

Effect of air-temperature and diet composition on the drying process of pellets for japanese abalone (Haliotis discus hannai) feeding

The aim of this research was to study the effect of air-temperature and diet composition on the mass transfer kinetics during the drying process of pellets used for Japanese Abalone (Haliotis discus hannai) feeding. In the experimental design, three temperatures were used for convective drying, as well as three different diet compositions (Diets A, B and C), in which the amount of fishmeal, spirulin, algae, fish oil and cornstarch varied. The water diffusion coefficient of the pellets was determined using the equation of Fick's second law, which resulted in values between 0.84-1.94×10-10 m²/s. The drying kinetics was modeled using Page, Modified Page, Root of time, Exponential, Logarithmic, Two-Terms, Modified Henderson-Pabis and Weibull models. In addition, two new models, referred to as 'Proposed' models 1 and 2, were used to simulate this process. According to the statistical tests applied, the models that best fitted the experimental data were Modified Henderson-Pabis, Weibull and Proposed model 2, respectively. Bifactorial analysis of variance ANOVA showed that Diet A (fishmeal 44%, spirulin 9%, fish oil 1% and cornstarch 36%) presented the highest diffusion coefficient values, which were favored by the temperature increase in the drying process.

Moisture sorption isotherms of fresh and blanched pumpkin (Cucurbita moschata)

Moisture desorption isotherms of fresh and heat blanched pumpkins (Cucurbita moschata) were determined at three temperatures (30, 50 and 70 °C), using the standard, static-gravimetric method. The GAB, Oswin, BET, Halsey, and Henderson models were tested and, with the exception of the Henderson model, showed satisfactory fits to the experimental data. The GAB model was used to analyze the fitting ability to describe the isotherm type. The shape of the desorption isotherms of fresh and blanched pumpkin at 30 and 50 °C was intermediate to types II and III, and at 70 °C it was of type II for the blanched pumpkin and close to type II for the fresh sample. The influence of blanching on the decrease in equilibrium moisture was very small compared to the fresh samples and it was related to the loss of soluble solids during the pre-treatment. The isosteric heat of sorption measures indicated that a larger amount of heat was required to remove the water from the fresh samples than from the blanched ones.