Search results for "EXPA"
showing 10 items of 820 documents
Increased Muscleblind levels by chloroquine treatment improve myotonic dystrophy type 1 phenotypes in in vitro and in vivo models
2019
Myotonic dystrophy type 1 (DM1) is a life-threatening and chronically debilitating neuromuscular disease caused by the expansion of a CTG trinucleotide repeat in the 3′ UTR of the DMPK gene. The mutant RNA forms insoluble structures capable of sequestering RNA binding proteins of the Muscleblind-like (MBNL) family, which ultimately leads to phenotypes. In this work, we demonstrate that treatment with the antiautophagic drug chloroquine was sufficient to up-regulate MBNL1 and 2 proteins in Drosophila and mouse (HSA LR ) models and patient-derived myoblasts. Extra Muscleblind was functional at the molecular level and improved splicing events regulated by MBNLs in all disease models. In vivo,…
Modelling the inhibitory effect of copper sulfate on the growth of Penicillium expansum and Botrytis cinerea
2011
Aims: This study aimed to investigate the effect of copper sulfate (from 0 to 8 mmol kg−1) on radial growth rate and lag time of two moulds responsible for vine grapes spoilage: Penicillium expansum strain 25·03 and Botrytis cinerea, strains BC1 and BC2. Methods and results: A new model was developed to describe tailing and shoulders in the inhibition curves. Because of tailing, the minimum inhibitory concentration (MIC), was not defined as the concentration at which no growth was observed, but as the concentration at which the lag time was infinite. The concentrations at which μ = μopt/2, (Cu50), were in the range of 2·2–2·6 mmol kg−1. Radial growth rate of P. expansum and the reciprocal…
Influence of temperature, copper and CO2on spore counts and geosmin production byPenicillium expansum
2012
Background and Aim: Musts and wines produced from rotten grapes often have an earthy/musty odour, with geosmin the responsible compound. Penicillium expansum is considered a potential source of geosmin in rotten grapes from vineyards treated with copper-based fungicides. Methods and Results: The laboratory study assessed the influence of temperature (10‐30°C), copper concentration (0‐76.50 mg/L) and CO 2 in the headspace (0.03‐3%) on the spore count and the production of geosmin by P. expansum according to a Doehlert design. The spore count and the production of geosmin (ng/mg biomass) were significantly correlated (r = 0.78). Copper had no significant effect on the spore count but was the …
El consentimiento en el proceso penal : ¿un oxímoron?
2021
We are involved in a moment of deep changes in Criminal Procedure due, among other reasons, to the permanent expansion of Criminal Law. Legislative modifications have taken place one another over the world and they have led to a functional reformulation of the role played by the protagonists of Criminal Procedure. These changes have not finished yet and the future is still to come, but new elements are already emerging: the consent of the actors of the process and of the victims and the reinforcement of the principle of opportunity are undoubtedly some of them. Probation, diversion, compliance, criminal mediation are institutions that stand on the principle of consent for procedural purpose…
Microbial community analysis, influence of reactor hybridation and effect of the proportion of glycol ethers/ethanol mixtures in EGSB reactors
2018
Antecedentes La emisión de compuestos orgánicos volátiles (COVs) puede causar diferentes problemas en la salud pública y en el medio ambiente, actuando como contaminantes primarios y permitiendo la formación de contaminantes secundarios como el ozono troposférico. Debido a estos problemas, la emisión de COVs está limitada en muchos países tales como EEUU o los pertenecientes a la Unión Europea, en este caso regulado por la directiva 2010/75/EU. Debido al uso de disolventes en su proceso productivo, la industria flexográfica es uno de los sectores que más contribuye a la emisión de este tipo de compuestos, y las técnicas biológicas han sido consideradas como una de las mejores disponibles pa…
Negative thermal expansion in cuprite-type compounds: A combined synchrotron XRPD, EXAFS, and computational study of Cu2O and Ag2O
2006
Cuprite-type oxides (Cu2O and Ag2O) are framework structures composed by two interpenetrated networks of metal-sharing M4O tetrahedra (M = Cu, Ag). Both compounds exhibit a peculiar negative thermal expansion (NTE) behaviour over an extended temperature range (9 240 K for Cu2O, 30-470 K for Ag2O). High-accuracy synchrotron powder diffraction and EXAFS measurements were performed from 10 K up to the decomposition temperature to understand the nature of the NTE effects. The critical comparison of the diffraction and absorption results concerning the temperature dependence of the interatomic distances and of the atomic vibrational parameters proves to be fundamental in defining the local dynam…
Between Interactions and Aggregates: The PolyQ Balance
2021
Abstract Polyglutamine regions (polyQ) are highly abundant consecutive runs of glutamine residues. They have been generally studied in relation to the so-called polyQ-associated diseases, characterized by protein aggregation caused by the expansion of the polyglutamine tract via a CAG-slippage mechanism. However, more than 4800 human proteins contain a polyQ, and only 9 of these regions are known to be associated with disease. Computational sequence studies and experimental structure determinations are completing a more interesting picture in which polyQ emerge as a motif for modulation of protein-protein interactions. But long polyQ regions may lead to an excess of interactions, and produc…
Characterization and Tuning of Ultra High Gradient Permanent Magnet Quadrupoles
2009
The application of quadrupole devices with high field gradients and small apertures requires precise control over higher order multipole field components. We present a new scheme for performance control and tuning, which allows the illumination of most of the quadrupole device aperture because of the reduction of higher order field components. Consequently, the size of the aperture can be minimized to match the beam size achieving field gradients of up to $500\text{ }\text{ }\mathrm{T}\text{ }{\mathrm{m}}^{\ensuremath{-}1}$ at good imaging quality. The characterization method based on a Hall probe measurement and a Fourier analysis was confirmed using the high quality electron beam at the M…
A series expansion of the extended Debye-H�ckel equation and application to linear prediction of stability constants
1996
The Debye-Hückel semiempirical extended equation is frequently used to calculate activity coefficients of chemical species and equilibrium constants at ionic strengths different from those used in their experimental evaluation. A series expansion of the extended Debye-Hückel equation is proposed here and checked with experimental data taken from the literature. The expansion is linear in the ionic parameters and yields a geometrical series which converges rapidly and that enables the accurate calculation of interpolated and extrapolated activity coefficients and equilibrium constants by simple and multiple linear regression without previous knowledge of the ionic parameters.
Neuromuscular and mobility responses to a vibration session in hypoxia in multiple sclerosis
2020
AbstractThe aim of this study was to investigate the acute effects of vibration training (WBVT) under hypoxic and normoxic conditions on the voluntary rate of force development (RFD), balance and muscle oxygen saturation (SMO2) in persons with Multiple Sclerosis (MS). 10 participants completed the study (30% males, 44.4±7.7 years, 164.3±8.9 cm, 65.2±11.1 kg, 2.5±1.3 Expanded Disability Status Scale, 24.1±4.0 kg.m−2 BMI). Maximal force, RFD during isometric knee extension, static balance with eyes open and closed and sit-to-stand test were evaluated before and immediately after one session of WBVT (12 60-s bout of vibration; frequency 35 Hz; amplitude 4 mm; 1-min rest intervals) under both n…