Quality of life and unmet needs in patients with fabry disease: a qualitative study.

Background: Patients with Fabry disease (FD) consider their quality of life to be significantly affected. The majority of studies evaluate the quality of life using quantitative measures and standardised scales that offer relevant information about experience with the disease in multiple aspects. The main objective of the research was to examine in depth the quality of life and unmet needs of patients diagnosed with FD in relation to their disease and treatment.

Neuropsychological rehabilitation in patients with relapsing-remitting multiple sclerosis: a systematic review.

Multiple sclerosis (MS) is an autoimmune, chronic, inflammatory and demyelinating disease that affects the Central Nervous System (CNS). It is the most common disabling neurological disease in young patients not caused by traumatic shock. Depending on how symptoms appear and how often they occur, there are different subtypes of MS.

Factors Associated with Therapeutic Adherence in Multiple Sclerosis in Spain.

Purpose: Adherence to disease-modifying therapies (DMTs) in multiple sclerosis (MS) is a complex and multidimensional phenomenon. Identifying the predictors of therapeutic adherence in MS will guide the design of interventions to improve health outcomes. Our aim was to assess the degree of adherence to pharmacological treatments, assess the relationship between patient-related factors and pharmacological adherence and to identify predictors of adherence to pharmacological treatments in patients with MS in Spain.

C. elegans expressing D76N β-microglobulin: a model for in vivo screening of drug candidates targeting amyloidosis.

The availability of a genetic model organism with which to study key molecular events underlying amyloidogenesis is crucial for elucidating the mechanism of the disease and the exploration of new therapeutic avenues. The natural human variant of β-microglobulin (D76N β-m) is associated with a fatal familial form of systemic amyloidosis. Hitherto, no animal model has been available for studying in vivo the pathogenicity of this protein.

A new method for microwave assisted ethanolic extraction of Mentha rotundifolia bioactive terpenoids.

A new microwave-assisted extraction (MAE) method using ethanol as solvent has been optimized by means of a Box-Behnken experimental design for the enhanced extraction of bioactive terpenoids from Mentha rotundifolia leaves; 100°C, 5 min, 1.125 g dry sample: 10 mL solvent and a single extraction cycle were selected as optimal conditions. Improved performance of MAE method in terms of extraction yield and/or reproducibility over conventional solid-liquid extraction and ultrasound assisted extraction was also previously assessed.

Multidisciplinary Consensus on the Nonadherence to Clinical Management of Inhaled Therapy in Spanish asthma patients.

Purpose: Rates of nonadherence to asthma treatment in Spain are between 24% and 76%, which results in poor disease control and increased health care costs. The main objective of this multidisciplinary consensus was to investigate the opinions of health professionals and patients regarding adherence to inhaled therapy in Spain. The results will help to identify the causes of nonadherence and to establish strategies to detect and correct the problem.

Enhanced toxicity of silver nanoparticles in transgenic Caenorhabditis elegans expressing amyloidogenic proteins.

The increasing number of applications of silver nanoparticles (AgNP) prompted us to assess their toxicity in vivo. We have investigated their effects on wild type and transgenic Caenorhabditis elegans (C. elegans) strains expressing two prototypic amyloidogenic proteins: β2-microglobulin and Aβ peptide3-42.

Impact of processing conditions on the kinetic of vitamin C degradation and 2-furoylmethyl amino acid formation in dried strawberries.

In this paper, a study on the usefulness of the determination of vitamin C together with indicators of the initial steps of Maillard reaction (2-furoylmethyl amino acids, 2-FM-AA) during the convective drying of strawberries has been carried out for the first time, paying special attention to the kinetics of degradation and formation, respectively, of both parameters. Formation of 2-FM-AA of Lys, Arg and GABA and vitamin C loss increased with time and temperature following, respectively, a zero and first-order kinetics. As supported by its lower activation energy, 2-FM-GABA (55.

C. elegans expressing human β2-microglobulin: a novel model for studying the relationship between the molecular assembly and the toxic phenotype.

Availability of living organisms to mimic key step of amyloidogenesis of human protein has become an indispensable tool for our translation approach aiming at filling the deep gap existing between the biophysical and biochemical data obtained in vitro and the pathological features observed in patients. Human β(2)-microglobulin (β(2)-m) causes systemic amyloidosis in haemodialysed patients. The structure, misfolding propensity, kinetics of fibrillogenesis and cytotoxicity of this protein, in vitro, have been studied more extensively than for any other globular protein.

Vitamin C content and sensorial properties of dehydrated carrots blanched conventionally or by ultrasound.

Vitamin C content and sensorial properties have been evaluated in air-dried carrots previously subjected to different ultrasound (US) or conventional blanching pretreatments. In addition, mass spectral fingerprints obtained by the Headspace ChemSensor System have been evaluated for the first time for classification of carrots according to their processing. Conventional blanching treatments at high temperature gave rise to carrots with retention of vitamin C in the range 37.

The two tryptophans of β2-microglobulin have distinct roles in function and folding and might represent two independent responses to evolutionary pressure.

Background: We have recently discovered that the two tryptophans of human β2-microglobulin have distinctive roles within the structure and function of the protein. Deeply buried in the core, Trp95 is essential for folding stability, whereas Trp60, which is solvent-exposed, plays a crucial role in promoting the binding of β2-microglobulin to the heavy chain of the class I major histocompatibility complex (MHCI). We have previously shown that the thermodynamic disadvantage of having Trp60 exposed on the surface is counter-balanced by the perfect fit between it and a cavity within the MHCI heavy chain that contributes significantly to the functional stabilization of the MHCI.

Essential oil composition of the different parts of Eryngium corniculatum Lam. (Apiaceae) from Spain.

The essential oil from the different parts (inflorescences, stems+leaves and roots) of E. corniculatum Lam. gathered in Guadalajara (Spain) has been extracted by steam distillation and analysed by gas chromatography and gas chromatography coupled to mass spectrometry.