252 research outputs found
Improving function of biocontrol agents incorporated in antifungal fruit coatings: a review
[EN] The in-field performance of microbial biocontrol agents (BCAs) against fungal pathogens in fruit is subject to considerable variability due to their sensitivity to both adverse environmental conditions and their fluctuations. Therefore, to achieve an adequate development and implementation of biological agent-based products, it is necessary to improve their resistance and ability to control fungal diseases under a wide range of conditions. In this review, an overview of the latest strategies for the enhancement of the action of BCAs is given. The combination of the antagonists with edible polymers able to form coatings is one of the approaches with the greatest potential and it is analysed in depth. This formulation approach of biocontrol products, including adequate microbial protectants, can yield stable products with high microbial viability, ready for field applications, with improved adherence and survival of the BCA once applied in plant. The most recent studies into this field are reviewed and summarised.This work was supported by Generalitat Valenciana [grant number VALi+d 2013]; Instituto Nacional de Investigación y TecnologÃa Agraria y Alimentaria [grant number RTA2012-00067-C02].MarÃn-Gozalbo, A.; Atarés Huerta, LM.; Chiralt, A. (2017). Improving function of biocontrol agents incorporated in antifungal fruit coatings: a review. Biocontrol Science and Technology. 27(10):1220-1241. doi:10.1080/09583157.2017.1390068S12201241271
Effect of pore generator on microstructure and resistivity of Sb2O3 and CuO doped SnO2 electrodes
[EN] Sb(2)O(3)and CuO doped SnO(2)ceramic electrodes could be an alternative to the ones currently used ones in the electrooxidation process of water pollutants. The rise of electrode surface by introducing a porogen agent on the composition was analysed in order to increase the electrochemical active surface. For this reason, several substances were tested. Although the densification and total pore volume had similar values, the microstructures and the pore size distributions generated were strongly dependent on porogen nature. A total of five porogens were tested, but petroleum coke turned out to be the best option for these electrodes. It was found that the electrical resistivity depends on the nature of pore generator. Furthermore, its relation to the porosity can be modelled with Archie's or Pabst's equations.The authors are very grateful to the Ministerio de Economia y Competitividad (Projects: CTQ2015-65202-C2-1-R and CTQ2015-65202-C2-2-R) and to the European Regional Development Fund (FEDER), for their economic support.Sánchez-Rivera, M.; Gozalbo, A.; Pérez-Herranz, V.; Mestre, S. (2020). Effect of pore generator on microstructure and resistivity of Sb2O3 and CuO doped SnO2 electrodes. Journal of Porous Materials. 27(6):1801-1808. https://doi.org/10.1007/s10934-020-00959-0S18011808276C.A. MartÃnez-Huitle, S. Ferro, Electrochemical oxidation of organic pollutants for the wastewater treatment: direct and indirect processes. Chem. Soc. Rev. 35, 1324–1340 (2006). https://doi.org/10.1039/B517632HC.A. Kent, J.J. Concepcion, C.J. Dares, D.A. Torelli, A.J. Rieth, A.S. Miller, P.G. Hoertz, T.J. Meyer, Water oxidation and oxygen monitoring by cobalt-modified fluorine-doped tin oxide electrodes. J. Am. 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Estudio del efecto fotocrómico que presentan los vidriados opacificados por esfena
White glazes opacified by sphene, based on the system SiO2-CaO-TiO2, are an alternative to the glazes in which the opacifying
phase is zircon. However, it has been observed that this type of glaze displays photochromism (reversible change of colour
after exposure to a light source), involving the appearance of a yellowish shade after the glaze has been subjected to sunlight
for several hours, which then disappears after a few days. This effect has led to limited use of this type of glaze.
In this study, a method of quantifying the photochromic effect by means of a UV light source is established. The microstructural
characterisation of glazes with a pronounced tendency to display a photochromic effect has allowed the possible causes of the
photochromic effect to be determined. Finally, a series of tests have been conducted that show that the photochromic effect is
related to the impurities present in the glaze, as well as to the type and quantity of crystalline phases contained in the glaz
Expanding the clinical spectrum of 3-phosphoglycerate dehydrogenase deficiency
3-Phosphoglycerate dehydrogenase (3-PGDH) deficiency is considered to be a rare cause of congenital microcephaly, infantile onset of intractable seizures and severe psychomotor retardation. Here, we report for the first time a very mild form of genetically confirmed 3-PGDH deficiency in two siblings with juvenile onset of absence seizures and mild developmental delay. Amino acid analysis showed serine values in CSF and plasma identical to what is observed in the severe infantile form. Both patients responded favourably to relatively low dosages of serine supplementation with cessation of seizures, normalisation of their EEG abnormalities and improvement of well-being and behaviour. These cases illustrate that 3-PGDH deficiency can present with mild symptoms and should be considered as a treatable disorder in the differential diagnosis of mild developmental delay and seizures. Synopsis: we present a novel mild phenotype in patients with 3-PGDH deficiency
Prediction of disease severity in multiple acyl-CoA dehydrogenase deficiency:a retrospective and laboratory cohort study
Multiple acyl-CoA dehydrogenase deficiency (MADD) is an ultra-rare inborn error of mitochondrial fatty acid oxidation (FAO) and amino acid metabolism. Individual phenotypes and treatment response can vary markedly. We aimed to identify markers that predict MADD phenotypes. We performed a retrospective nationwide cohort study; then developed an MADD-disease severity scoring system (MADD-DS3) based on signs and symptoms with weighed expert opinions; and finally correlated phenotypes and MADD-DS3 scores to FAO flux (oleate and myristate oxidation rates) and acylcarnitine profiles after palmitate loading in fibroblasts. Eighteen patients, diagnosed between 1989 and 2014, were identified. The MADD-DS3 entails enumeration of eight domain scores, which are calculated by averaging the relevant symptom scores. Lifetime MADD-DS3 scores of patients in our cohort ranged from 0 to 29. FAO flux and [U-13C]C2-, C5-, and [U-13C]C16-acylcarnitines were identified as key variables that discriminated neonatal from later onset patients (all P <.05) and strongly correlated to MADD-DS3 scores (oleate: r = −.86; myristate: r = −.91; [U-13C]C2-acylcarnitine: r = −.96; C5-acylcarnitine: r =.97; [U-13C]C16-acylcarnitine: r =.98, all P <.01). Functional studies in fibroblasts were found to differentiate between neonatal and later onset MADD-patients and were correlated to MADD-DS3 scores. Our data may improve early prediction of disease severity in order to start (preventive) and follow-up treatment appropriately. This is especially relevant in view of the inclusion of MADD in population newborn screening programs
Fertility preservation in female classic galactosemia patients
Almost every female classic galactosemia patient develops primary ovarian insufficiency (POI) as a diet-independent complication of the disease. This is a major concern for patients and their parents, and physicians are often asked about possible options to preserve fertility. Unfortunately, there are no recommendations on fertility preservation in this group. The unique pathophysiology of classic galactosemia with a severely reduced follicle pool at an early age requires an adjusted approach. In this article recommendations for physicians based on current knowledge concerning galactosemia and fertility preservation are made. Fertility preservation is only likely to be successful in very young prepubertal patients. In this group, cryopreservation of ovarian tissue is currently the only available technique. However, this technique is not ready for clinical application, it is considered experimental and reduces the ovarian reserve. Fertility preservation at an early age also raises ethical questions that should be taken into account. In addition, spontaneous conception despite POI is well described in classic galactosemia. The uncertainty surrounding fertility preservation and the significant chance of spontaneous pregnancy warrant counseling towards conservative application of these techniques. We propose that fertility preservation should only be offered with appropriate institutional research ethics approval to classic galactosemia girls at a young prepubertal age
Parasitosis intestinales en escolares de la ciudad de Valencia. Encuesta de prevalencia.
Fundamento: La importancia de la lucha contra las infecciones intestinales entre las que se incluyen las parasitarias radica en su alta prevalencia y su distribución mundial. El objeto del estudio es conocer la prevalencia de parásitos en heces en la población escolar de la ciudad de Valencia y se justifica en el desconocimiento de la endemia parasitaria, combinada con los importantes cambios demográficos y sociales por el fenómeno inmigratorio.Material y método: Se realiza un estudio descriptivo, de corte transversal con distribución de características y atributos de los sujetos. Muestra asignada por conglomerados entre la población (64.200 alumnos) asistente a centros de Educación Infantil (3-5 años) y Primaria (6-11 años) segmentada por estacionalidad. Tamaño muestral 1.031 niños, prevalencia esperada 21,8% y error de muestreo 2,5%. Se aplicó encuesta auto-cumplimentada y toma de muestra única de heces: bote de recogida con fijador y cinta de Graham para detección de entero-parásitos. Resultados: En el segmento estacional primavera-otoño se recuperaron 523 sujetos, (29,8% son población inmigrante). La prevalencia general de infección fue 27,4%. (IC95%: 23,58-31,22), que alcanza el 40,52% (IC95%: 32,5-47,8) en inmigrantes, RP: 2,45 (IC95%: 1,64-3,67, p<0,0001). Se detectan 11 especies entero-parasitarias, la más frecuente Blastocystis hominis: 14,9%, de los que 57,33% corresponden a escolares inmigrantes RP: 3,16 (IC95%: 2,08-4,79, p<0,0001). Al comparar el origen de los infectados por ciclos escolares se observan diferencias en E.Primaria que se acentúan con la edad, OR1ºciclo: 2,6 (IC95%: 1,2-5,4), OR2ºciclo: 3,15 (IC95%: 1,3-7,3), OR3ªciclo: 6,44 (IC95%: 2,05-20,18). No se observan diferencias por género ni globalmente ni entre los inmigrantes.Conclusiones: La prevalencia general de parasitación observada resulta relevante. En general los datos conocidos coinciden con nuestra encuesta en que el 89% de las parasitosis son por protozoos. Los nematodos suponen un 9% y, de ellos, el más frecuente es E vermicularis. Con otros trabajos consultados, realizados en los años 90 se comprueba un cambio del patrón entero-parasitario y responde al cambio sociodemográfico producido por la inmigración. La fuerza de asociación y la relación infectados/edad en niños inmigrantes pone de manifiesto la necesidad de una especial vigilancia
Allelic Expression Imbalance Promoting a Mutant PEX6 Allele Causes Zellweger Spectrum Disorder
Zellweger spectrum disorders (ZSDs) are autosomal-recessive disorders that are caused by defects in peroxisome biogenesis due to bi-allelic mutations in any of 13 different PEX genes. Here, we identified seven unrelated individuals affected with an apparent dominant ZSD in whom a heterozygous mutant PEX6 allele (c.2578C>T [p.Arg860Trp]) was overrepresented due to allelic expression imbalance (AEI). We demonstrated that AEI of PEX6 is a common phenomenon and is correlated with heterozygosity for a frequent variant in the 3' untranslated region (UTR) of the mutant allele, which disrupts the most distal of two polyadenylation sites. Asymptomatic parents, who were heterozygous for PEX c.2578C>T, did not show AEI and were homozygous for the 3' UTR variant. Overexpression models confirmed that the overrepresentation of the pathogenic PEX6 c.2578T variant compared to wild-type PEX6 c.2578C results in a peroxisome biogenesis defect and thus constitutes the cause of disease in the affected individuals. AEI promoting the overrepresentation of a mutant allele might also play a role in other autosomal-recessive disorders, in which only one heterozygous pathogenic variant is identified.</p
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