A Poglut1 mutation causes a muscular dystrophy with reduced Notch signaling and satellite cell loss

Skeletal muscle regeneration by muscle satellite cells is a physiological mechanism activated upon muscle damage and regulated by Notch signaling. In a family with autosomal recessive limbgirdle muscular dystrophy, we identified a missense mutation in POGLUT1 (protein O-glucosyltransferase 1), an enzyme involved in Notch posttranslational modification and function. In vitro and in vivo experiments demonstrated that the mutation reduces Oglucosyltransferase activity on Notch and impairs muscle development. Muscles from patients revealed decreased Notch signaling, dramatic reduction in satellite cell pool and a muscle-specific adystroglycan hypoglycosylation not present in patients’ fibroblasts. Primary myoblasts from patients showed slow proliferation, facilitated differentiation, and a decreased pool of quiescent PAX7+ cells. A robust rescue of the myogenesis was demonstrated by increasing Notch signaling. None of these alterations were found in muscles from secondary dystroglycanopathy patients. These data suggest that a key pathomechanism for this novel form of muscular dystrophy is Notch-dependent loss of satellite cells.Junta de Andalucía PI-0017-201

Obra Gráfica: Helena Gómez Serrano De la naturaleza, un paisaje interior.

Resumen Desde que el hombre se reconoce como tal, existe en su interior una necesidad por representar su entorno. Los vestigios encontrados en las cuevas de Altamira son apenas una confirmación de esta relación

Electrical conductivity of metal (hydr)oxide–activated carbon composites under compression. A comparison study

[EN]From a granular commercial activated carbon (AC) and six metal (hydr)oxide precursors, including Al(NO3)3, Fe(NO3)3, SnCl2, TiO2, Na2WO4 and Zn(NO3)2, a broadly varied series of metal (hydr)oxideeAC composites were prepared by wet impregnation and subsequent oven-drying at 120 C. Here, the electrical conductivity of the resulting products was studied under moderate compression. The influence of the applied pressure, sample volume, mechanical work, and density of the hybrid materials was thoroughly investigated. The dc electrical conductivity of the compressed samples was measured at room temperature by the four-probe method. Compaction assays show that the mechanical properties of the composites are largely determined by the carbon matrix. Both the decrease in volume and the increase in density under compression were very small and only significant at pressures lower than 100 kPa for AC and most composites. By contrast, the bulk electrical conductivity of the hybrid materials was strongly influenced by the nature, content and intrinsic conductivity of the supported metal phases, which act as insulating thin layers thereby hindering the effective electron transport between AC cores of neighbouring sample particles in contact under compression. Conductivity values for the composites were lower than for the raw AC, all of them falling in the range of typical semiconductor materials. The patterns of variation of the electrical conductivity with pressure and mechanical work were slightly similar, thus suggesting the predominance of the pressure effects rather than the volume one

A Stable High-Capacity Lithium-Ion Battery Using a Biomass-Derived Sulfur-Carbon Cathode and Lithiated Silicon Anode

A full lithium-ion-sulfur cell with a remarkable cycle life was achieved by combining an environmentally sustainable biomass-derived sulfur-carbon cathode and a pre-lithiated silicon oxide anode. X-ray diffraction, Raman spectroscopy, energy dispersive spectroscopy, and thermogravimetry of the cathode evidenced the disordered nature of the carbon matrix in which sulfur was uniformly distributed with a weight content as high as 75 %, while scanning and transmission electron microscopy revealed the micrometric morphology of the composite. The sulfur-carbon electrode in the lithium half-cell exhibited a maximum capacity higher than 1200 mAh gS−1, reversible electrochemical process, limited electrode/electrolyte interphase resistance, and a rate capability up to C/2. The material showed a capacity decay of about 40 % with respect to the steady-state value over 100 cycles, likely due to the reaction with the lithium metal of dissolved polysulfides or impurities including P detected in the carbon precursor. Therefore, the replacement of the lithium metal with a less challenging anode was suggested, and the sulfur-carbon composite was subsequently investigated in the full lithium-ion-sulfur battery employing a Li-alloying silicon oxide anode. The full-cell revealed an initial capacity as high as 1200 mAh gS−1, a retention increased to more than 79 % for 100 galvanostatic cycles, and 56 % over 500 cycles. The data reported herein well indicated the reliability of energy storage devices with extended cycle life employing high-energy, green, and safe electrode materials

Algunos conceptos para la mejora de la rentabilidad en explotaciones cunícolas

La cunicultura no atraviesa por buenos momentos y la rentabilidad de muchas de las explotaciones españolas está cada vez más comprometida. En este trabajo se intenta conocer y razonar sobre algunas cuestiones generales que podrían contribuir a la mejora de la rentabilidad de las explotaciones cunícolas. El conocimiento sobre el origen de los ingresos y costes de nuestra explotación tanto fijos como variables es imprescindible a la hora de adoptar medidas adecuadas que contribuyan a mejorar la situación económica de nuestras explotaciones

Analysis of the drinks that contribute to the hydration of andalusian sportspeople

AbstractObjectivesTo estimate the water balance in a healthy population of sportspeople from Southern Spain and determine the sources of their fluid intake, evaluating the contribution of different types of drink and comparing the results by sex and province of residence.MethodsThree hundred eighty-six sportspeople (231 males, 152 females) were enrolled in the study. A questionnaire was administered to calculate nutrient intake through diet and physical activity, and anthropometric measures were taken according to ISAK standards. SPSS-15 was used for data analyses.ResultsFruit juice, tap water, bottled water, processed fruit juice, carbonated drinks, and isotonic drinks comprised 96% of the total water intake. Simple sugar consumption represented 4.44% of daily calorie intake. Significant differences were found between sexes and between professionals and amateurs. The amount of drinks consumed varied as a function of the quality of the drinking water, which significantly differed among the eight Andalusian provinces.ConclusionThis study population did not fully meet fluid intake recommendations, compliance with hydration recommendations varied as a function of the sex and the amateur or professional status of these sportspeople. The pattern of drinks consumption also differed according to their place of residence

Deciphering the Role and Signaling Pathways of PKCα in Luminal A Breast Cancer Cells.

Protein kinase C (PKC) comprises a family of highly related serine/threonine protein kinases involved in multiple signaling pathways, which control cell proliferation, survival, and differentiation. The role of PKCα in cancer has been studied for many years. However, it has been impossible to establish whether PKCα acts as an oncogene or a tumor suppressor. Here, we analyzed the importance of PKCα in cellular processes such as proliferation, migration, or apoptosis by inhibiting its gene expression in a luminal A breast cancer cell line (MCF-7). Differential expression analysis and phospho-kinase arrays of PKCα-KD vs. PKCα-WT MCF-7 cells identified an essential set of proteins and oncogenic kinases of the JAK/STAT and PI3K/AKT pathways that were down-regulated, whereas IGF1R, ERK1/2, and p53 were up-regulated. In addition, unexpected genes related to the interferon pathway appeared down-regulated, while PLC, ERBB4, or PDGFA displayed up-regulated. The integration of this information clearly showed us the usefulness of inhibiting a multifunctional kinase-like PKCα in the first step to control the tumor phenotype. Then allowing us to design a possible selection of specific inhibitors for the unexpected up-regulated pathways to further provide a second step of treatment to inhibit the proliferation and migration of MCF-7 cells. The results of this study suggest that PKCα plays an oncogenic role in this type of breast cancer model. In addition, it reveals the signaling mode of PKCα at both gene expression and kinase activation. In this way, a wide range of proteins can implement a new strategy to fine-tune the control of crucial functions in these cells and pave the way for designing targeted cancer therapies.Work in Murcia was supported by grants BFU2017-87222-P (MICINN, Spain-FEDER) to S.C.-G. and J.C.G.-F. and Fundación Séneca Region de Murcia 20885/PI/18 to S.C.-G.S

Propuesta de restauración y puesta en valor patrimonial de la ex hacienda San Agustín en el municipio de Jamay, Jalisco

ITESO, A.C

Morphometric and microstructural characteristics of hippocampal subfields in mesial temporal lobe epilepsy and their correlates with mnemonic discrimination.

Pattern separation (PS) is a fundamental aspect of memory creation that defines the ability to transform similar memory representations into distinct ones, so they do not overlap when storing and retrieving them. Experimental evidence in animal models and the study of other human pathologies have demonstrated the role of the hippocampus in PS, in particular of the dentate gyrus (DG) and CA3. Patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HE) commonly report mnemonic deficits that have been associated with failures in PS. However, the link between these impairments and the integrity of the hippocampal subfields in these patients has not yet been determined. The aim of this work is to explore the association between the ability to perform mnemonic functions and the integrity of hippocampal CA1, CA3, and DG in patients with unilateral MTLE-HE. To reach this goal we evaluated the memory of patients with an improved object mnemonic similarity test. We then analyzed the hippocampal complex structural and microstructural integrity using diffusion weighted imaging. Our results indicate that patients with unilateral MTLE-HE present alterations in both volume and microstructural properties at the level of the hippocampal subfields DG, CA1, CA3, and the subiculum, that sometimes depend on the lateralization of their epileptic focus. However, none of the specific changes was found to be directly related to the performance of the patients in a pattern separation task, which might indicate a contribution of various alterations to the mnemonic deficits or the key contribution of other structures to the function. we established for the first time the alterations in both the volume and the microstructure at the level of the hippocampal subfields in a group of unilateral MTLE patients. We observed that these changes are greater in the DG and CA1 at the macrostructural level, and in CA3 and CA1 in the microstructural level. None of these changes had a direct relation to the performance of the patients in a pattern separation task, which suggests a contribution of various alterations to the loss of function