Evolution of the retinal function by flash-ERG in one child suffering from neuronal ceroid lipofuscinosis CLN2 treated with cerliponase alpha: case report.

Introduction: Neuronal ceroid lipofuscinoses (CLN) are neurodegenerative disorders among the most frequent, inherited as an autosomal recessive trait. Affected patients can present with progressive decline in cognitive and motor functions, seizures, a shortened life span and visual deficiency. CLN2 is one of the rare CLN that benefits from treatment by cerliponase alpha an enzyme replacement therapy.

Preparation and Characterization of Highly Elastic Foams with Enhanced Electromagnetic Wave Absorption Based on Ethylene-Propylene-Diene-Monomer Rubber Filled with Barium Titanate/Multiwall Carbon Nanotube Hybrid.

Hybrid ethylene-propylene-diene-monomer (EPDM) nanocomposite foams were produced via compression molding with enhanced electromagnetic wave absorption efficiency. The hybrid filler, consisting of 20 phr ferroelectric barium titanate (BT) and various loading fractions of multi-wall carbon nanotubes (MWCNTs), synergistically increased the electromagnetic (EM) wave absorption characteristics of the EPDM foam. Accordingly, while the EPDM foam filled with 20 phr BT was transparent to the EM wave within the frequency range of 8.

Pulmonary Alterations Among Workers in a Dental Prosthesis Laboratory: Exploring High Dust Concentrations and Novel Findings of Bacterial Genera in the Workplace to Achieve Improved Control.

Objective: To evaluate the pulmonary alterations in workers from a dental prosthesis laboratory and explore dust and bacterial dissemination generated in the laboratory.

Methods: Spirometry and computerized axial tomography were performed on 67 workers. Dust in workplace air was determined using the filtration-gravimetric method, and bacterial detection was explored using 16S rDNA gene sequencing.

Highly Deformable Porous Electromagnetic Wave Absorber Based on Ethylene-Propylene-Diene Monomer/Multiwall Carbon Nanotube Nanocomposites.

The need for electromagnetic interference (EMI) shields has risen over the years as the result of our digitally and highly connected lifestyle. This work reports on the development of one such shield based on vulcanized rubber foams. Nanocomposites of ethylene-propylene-diene monomer (EPDM) rubber and multiwall carbon nanotubes (MWCNTs) were prepared via hot compression molding using a chemical blowing agent as foaming agent.

Hafnium(IV) Chloride Catalyzes Highly Efficient Acetalization of Carbonyl Compounds.

Background: Hafnium(IV) tetrachloride efficiently catalyzes the protection of a variety of aldehydes and ketones, including benzophenone, acetophenone, and cyclohexanone, to the corresponding dimethyl acetals and 1,3-dioxolanes, under microwave heating. Substrates possessing acid-labile protecting groups (TBDPS and Boc) chemoselectively generated the corresponding acetal/ketal in excellent yields.

Aims And Objectives: In this study.

Paediatric-onset neuronal ceroid lipofuscinosis: first symptoms and presentation at diagnosis.

Neuronal ceroid lipofuscinoses (NCLs) are rare, progressive disorders. Through this series of 20 patients with NCL, we illustrate differences between subtypes in their presenting symptoms and clinical, imaging, and electrophysiological results to raise awareness of symptom diversity. Data were available on presenting symptoms, genetics, magnetic resonance imaging (MRI), electroencephalography (including with low-frequency intermittent photic stimulation), visual responses, and electron microscopy.

Transport Properties of One-Step Compression Molded Epoxy Nanocomposite Foams.

Owing to their high strength and stiffness, thermal and environmental stability, lower shrinkage, and water resistance, epoxy resins have been the preferred matrix for the development of syntactic foams using hollow glass microspheres. Although these foams are exploited in multiple applications, one of their issues is the possibility of breakage of the glass hollow microspheres during processing. Here, we present a straightforward and single-step foaming protocol using expandable polymeric microspheres based on the well-established compression molding process.

The three-layer technique for immediate implants on teeth without a buccal bone wall: a case report.

The buccal bone wall is the part of the socket of an anterior tooth that is most susceptible to resorption. Immediate implants offer advantages in terms of time, comfort, and esthetics, especially regarding the maintenance of the papillae architecture. However, the loss of the buccal bone wall is often a limitation for such a therapy.

Osseointegration of zirconia dental implants in animal investigations: A systematic review and meta-analysis.

Objective: To determine the osseointegration rate of zirconium dioxide (ZrO) dental implants in preclinical investigations.

Data: Data on the osseointegration rate was extracted considering the bone to implant contact (BIC), removal torque analysis (RTQ) and push-in tests. Meta analyses were conducted using multilevel multivariable mixed-effects linear regression models.

Frailty Is Associated With Lower Expression of Genes Involved in Cellular Response to Stress: Results From the Toledo Study for Healthy Aging.

Background: Specific mechanisms underlying frailty syndrome are not well known. Frailty can be viewed as a loss of functional reserve resulting in increased vulnerability to stressors. We hypothesize that pathways regulating cellular response to stress are potential players in the development of frailty.

Myoclonic jerks are commonly associated with absence seizures in early-onset absence epilepsy.

Typical absence seizures are observed in various epilepsy syndromes, however, few series have focused on early-onset absence epilepsy (EOAE). We aimed to evaluate the occurrence of this seizure type in children under 4 years of age in order to evaluate their electroclinical characteristics and outcome. We retrospectively studied (2006-2014) the electroclinical features of children with normal development and typical absence seizures starting before the age of 4 (with available pre-treatment video-EEG).

Simultaneous detection of two breast cancer-related miRNAs in tumor tissues using p19-based disposable amperometric magnetobiosensing platforms.

A novel magnetobiosensing approach for the rapid and simultaneous detection of two breast cancer-related miRs (miR-21 and miR-205) is reported. It involves the use of antimiR-21 and antimiR-205 specific probes, chitin-modified magnetic beads (Chitin-MBs), the p19 viral protein as capture bioreceptor and amperometric detection with the H2O2/hydroquinone (HQ) system at dual screen-printed carbon electrodes (SPdCEs). The use of SPdCEs allows the simultaneous independent amperometric readout for each target miR to be measured.

FM19G11 reverses endothelial dysfunction in rat and human arteries through stimulation of the PI3K/Akt/eNOS pathway, independently of mTOR/HIF-1α activation.

Background And Purpose: FM19G11 up-regulates mammalian target of rapamycin (mTOR)/hypoxia inducible factor-1α (HIF-1α) and PI3K/Akt pathways, which are involved in endothelial function. We evaluated the effects of FM19G11 on defective endothelial vasodilatation in arteries from rats and humans and investigated the mechanisms involved.

Experimental Approach: Effects of chronic in vivo administration of FM19G11 on aortic endothelial vasodilatation were evaluated together with ex vivo treatment in aortic and mesenteric arteries from control and insulin-resistant rats (IRR).

Magnetobiosensors based on viral protein p19 for microRNA determination in cancer cells and tissues.

MicroRNAs (miRs) have emerged as important clinical biomarkers with both diagnostic and prognostic value for relevant diseases, such as cancer. MiRs pose unique challenges for detection and are currently detected by northern blotting, real-time PCR, and microarray techniques. These expensive, complicated, and time-consuming techniques are not feasible for on-site miR determination.

[A retrospective cohort study of patients diagnosed of thyroid cancer in the southwest Madrid area. Predictive factors in differentiated thyroid cancer].

Objectives: To analyze the clinical and histopathological features of patients with thyroid cancer in the southwest Madrid area and to identify poor prognostic factors in the subgroup with differentiated thyroid carcinoma (DTC) of the follicular epitelium.

Patients And Methods: A retrospective cohort study of patients diagnosed with thyroid cancer at our hospital from 1998 to 2009. Significant clinical, surgical, and histopathological variables were included in Cox proportional hazard and logistic regression models to identify baseline factors predicting for death, recurrence, and persistent disease in DTC.

Glial activation in a pilocarpine rat model for epileptogenesis: a morphometric and quantitative analysis.

In this work we examined the correlation between long-term glial resilience and slow epileptogenesis using the pilocarpine-insult rat model. We assessed, quantitatively and morphometrically, glial fibrillary acidic protein (GFAP) expression and cell densities in hippocampus in a dose-response manner 2, 4 and 8 weeks after the pilocarpine insult. GFAP changes were correlated with observations on microglial activation.

Goshawk prey have more bacteria than non-prey.

1. Predators often prey on individuals that are sick or otherwise weakened. Although previous studies have shown higher abundance of parasites in prey, whether prey have elevated loads of micro-organisms remains to be determined.

The NMR structure and dynamics of the two-domain tick carboxypeptidase inhibitor reveal flexibility in its free form and stiffness upon binding to human carboxypeptidase B.

The structure of the tick carboxypeptidase inhibitor (TCI) and its backbone dynamics, free and in complex with human carboxypeptidase B, have been determined by NMR spectroscopy. Although free TCI has the same overall fold as that observed in the crystal structures of its complexes with metallocarboxypeptidase types A and B, there are structural differences at the linker between the two domains. The linker residues have greater flexibility than the globular domains, and the C-terminal residues are highly flexible in free TCI.

Solution structure and NMR characterization of the binding to methylated histone tails of the plant homeodomain finger of the tumour suppressor ING4.

Plant homeodomain (PHD) fingers are frequently present in proteins involved in chromatin remodelling, and some of them bind to histones. The family of proteins inhibitors of growth (ING) contains a PHD finger that bind to histone-3 trimethylated at lysine 4, and those of ING1 and ING2 also act as nuclear phosphoinositide receptors. We have determined the structure of ING4 PHD, and characterised its binding to phosphoinositides and histone methylated tails.

[Physiopathologic changes in brain death. Their importance for management and organ donation decisions].

Technological advances in the medical area have allowed for development of useful techniques to treat patients with diverse diseases. For example, at intensive care units this technology allows maintenance blood flow and tissue oxygenation even when brain death (BD) is already established and the individual cannot function. The function of heart, lungs, and other organs can be maintained with different devices, but maintenance of cerebral functions is not not yet possible.

[Incidence of gestational diabetes mellitus according to different diagnostic criteria in the southeast Madrid area. Influence of diagnosis on materno-fetal parameters].

BACKGROUND. In 1997, The Fourth International Workshop-Conference on Gestational Diabetes Mellitus (GDM) recommended the use of Carpenter and Coustan diagnostic criteria instead of those of Sullivan and Mahan used until then. The objective of this work was to study the incidence of GDM in the southeast Madrid area by applying both the classical and new criteria, as well as to determine whether patients diagnosed of GDM by the new criteria present a higher materno-fetal morbidity than treated GDM patients or non diabetic pregnant patients.

Towards understanding a molecular switch mechanism: thermodynamic and crystallographic studies of the signal transduction protein CheY.

The signal transduction protein CheY displays an alpha/beta-parallel polypeptide folding, including a highly unstable helix alpha4 and a strongly charged active site. Helix alpha4 has been shown to adopt various positions and conformations in different crystal structures, suggesting that it is a mobile segment. Furthermore, the instability of this helix is believed to have functional significance because it is involved in protein-protein contacts with the transmitter protein kinase CheA, the target protein FliM and the phosphatase CheZ.

Oncogenic Ras-induced germinal vesicle breakdown is independent of phosphatidylinositol 3-kinase in Xenopus oocytes.

A number of reports have identified phosphatidylinositol 3-kinase as a downstream effector of Ras in various cellular settings, in contrast to others supporting the notion that phosphatidylinositol 3-kinase acts upstream of Ras. Here, we used Xenopus oocytes, a model of Ras-mediated cell cycle progression (G2/M transition) to analyze the contribution of phosphatidylinositol 3-kinase to insulin/Ras-dependent signaling pathways leading to germinal vesicle breakdown and to ascertain whether phosphatidylinositol 3-kinase acts upstream or downstream of Ras in those signaling pathways. We analyzed the process of meiotic maturation induced by progesterone, insulin or micro-injected oncogenic Ras (Lys12) proteins in the presence and absence of specific inhibitors of phosphatidylinositol 3-kinase activity.

Structure analysis of two CheY mutants: importance of the hydrogen-bond contribution to protein stability.

The crystal structures of two double mutants (F14N/V21T and F14N/V86T) of the signal transduction protein CheY have been determined to a resolution of 2.4 and 2.2 A, respectively.

Amide hydrogen exchange and internal dynamics in the chemotactic protein CheY from Escherichia coli.

The backbone internal dynamics of the wild-type 129 amino acid alpha/beta parallel protein CheY and its double mutant F14N/P110G are analysed here by the hydrogen-exchange method. The F14N mutation is known to stabilise the protein and to accelerate refolding while P110G is destabilising and accelerates unfolding. We first assigned and characterised the double mutant by nuclear magnetic resonance (NMR), to try and discover any possible conformational change induced by the two mutations.