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dermic-derived fibroblasts for the study of amyotrophic lateral sclerosis

Neural Regeneration Research 2020年第11期15卷 2043-2044页

作者： Javier Riancho Sara Arozamena adolfo lopez de munain Service of Neurology Hospital Sierrallana-IDIVALTorrelavegaSpain department of Medicine and Psychiatry University of CantabriaSantanderSpain Centro de Investigacion en Red de Enfermedades Neurodegenerativas Instituto Carlos IIIMadridSpain Neurosciences Area Biodonostia Research InstituteService of NeurologyHospital Universitario DonostiaDepartment of NeurosciencesSchool of Medicine and NurseryUniversity of the Basque CountrySan SebastianSpain

Among neurodegenerative diseases,amyotrophic lateral sclerosis(ALS)is the most frequent one involving motor neurons(MNs).ALS incidence varies throughout the world ranging from 0.7 to 4 cases per 100,000 habitants and year(Riancho et al.,2016).This disease,which currently lacks an effective therapy,is characterized by a variable combination of upper and lower MN degeneration,leading to progressive muscle wasting which usually results in a terminal respiratory failure within 3 years after symptom onset(Zufiria et al.,2016).A small proportion of ALS cases show familial *** are related to mutations in specific causative genes(Cr9ORF72,TARDBP,FUS,SOD1 and others)which directly determine disease onset in *** contrast,more than 90 percent of cases are considered to be sporadic,in which generally unknown environmental and internal factors interact with genetic predisposing factors finally leading to disease(Riancho et al.,2018).From a histopathological point of view,ALS is characterized by MN damage and *** from ALS sporadic and most of familial patients exhibit prominent transactive response DNA-binding protein 43(TDP-43)cytoplasmic aggregates which are considered as the“pathological hallmark”of the disease,excepting those related to SOD1 and FUS mutations(Zufiria et al.,2016).TDP-43 is a DNA/RNA binding protein encoded by the TARDBP gene that controls the expression of many different genes.

关键词： al. amyotrophic degeneration

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Parkin and <i>LRRK2</i>/Dardarin Mutations in Early Onset Parkinson’s Disease in the Basque Country (Spain)

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Journal of Behavioral and Brain Science 2015年第3期5卷 101-108页

作者： J. F. Martí Massó J. Ruiz-Martínez C. Paisán-Ruiz A. Gorostidi A. Bergareche A. lopez de munain A. Alzualde J. Pérez-Tur Servicio Neurologia Hospital Donostia San Sebastián Spain Center for Biomedical Research in Neurodegenerative Diseases Network (CIBERNED) Spain Neurosciences Area Biodonostia Institute San Sebastián Spain department of Neuroscience University of Basque Country UPV-EHU San Sebastián Spain department of Genetics and Genomic Sciences Icahn School of Medicine at Mount Sinai New York USA Molecular Genetics Unit Instituto de Biomedicina de Valencia-CSIC Valencia Spain

We have performed a complete screening of the Parkin gene (PRKN2) and looked for ***2019Ser (G2019S) and ***1441Gly (R1441G) LRRK2/dardarin gene mutations in twenty seven patients with Parkinson’s disease (PD) with an age at onset younger than 50 years (EOPD), living in Gipuzkoa (Basque Country, Spain). Thirteen of them (48%) were PRKN2 mutation carriers. The c.255-256delA mutation was the most frequent, followed by a deletion involving exons 3 and 4. A deletion involving exons 3 and 12 of the PRKN2 gene and R1441G LRRK2 mutation was found together in one PD patient. Four out of fourteen PRKN2 negative patients carried the p.G2019S mutation. Both PRKN2 mutation carriers and non-carriers presented frequently with family history (10 PRKN2 mutation carriers and 8 PRKN2 non-carriers);in fact, five patients without a known gene mutation had a first degree relative affected, suggesting another monogenic disease. PRKN2 carriers presented with a younger age at onset (36.7 vs. 41.7) and more benign disease progression. Indeed, those PD patients younger than forty who initially presented with unilateral tremor became shortly bilateral. Relatively, symmetric parkinsonism and slow disease progression carried more frequently PRKN2 mutations than patients with unilateral akinetic rigid parkinsonism and age at onset later than 40 years. As expected in a recessive disease, PRKN2 patients present more often with affected siblings and unaffected patients. The G2019S LRRK2 mutation, less prevalent than R1441G in our area, may be also a frequent cause of PD in EOPD (4 patients).

关键词： Parkin Early Onset Parkinsonism Parkinson’s Disease LRRK2 Dardarin

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Exhaust Ventilation through a Solar Chimney： A Response to High Hermeticity in Welfare Housing

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Journal of Civil Engineering and Architecture 2015年第7期9卷 767-774页

作者： Marcos Eduardo Gonzalez-Trevizo adolfo Gomez-Amador Anibal Luna-Leon Carlos Escobar-del POZO Carlos Javier Esparza lopez Facultad de Arquitectura (Faculty of Architecture) Universidad Michoacana de San Nicoldls de Hidalgo (Michoacan Universityof Saint Nicholas of Hidalgo) Morelia MN 58030 Mexico Facultad de Arquitectura y DiseFzo (Faculty of Architecture and design) Universidad de Colima (University of Colima) ColimaCL 28400 Mexico Facultad de Arquitectura y Diseho (Faculty of Architecture and design) Universidad Aut6noma de Baja California (BajaCalifornia Autonomous University) Mexieali BC 21280 Mexico Facultad de Ingenieria Mecnica y Elctrica (Faculty of Mechanical and Electrical Engineering) Universidad de ColimaColima CL 28400 Mexico

This research reports on potentiality in the solar chimney as an exhaust ventilation device through a hypothetical statement that convenient weather conditions exist on sub-humid warm climate to enhance the performance ofa SC （solar chimney）, in order to increase ventilation to dissipate metabolic heat from inhabitants of airtight buildings. The methodology used in this research integrates the use of simplified mathematical models that predict in a semi-empirical way a potential volume flow through estimation of kinetic power generation, by natural circulation and stack effect in a typical covered-plate air solar collector. The study was carried out during the warm month of April, an important warm season for Colima, where the prevailing wind is about 6.8 m/s, the average temperature of maximums and minimums oscillates between 15.4℃and 34.4 ℃ with average relative humidity of 65% according to national weather service. A scale model of the SC was installed on environmental chambers. The results showed that this device is capable of generating ventilation conditions around light breeze parameters near between 0.6 m/s and 1.5 m/s width, a better performance at day in 45% approximately than the night values, making ventilation rates up 0.24 ACH （air changes per hour） as average air change rate.

关键词： Exhaust ventilation solar chimney air solar collector welfare housing.

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Precision medicine in sepsis and septic shock:From omics to clinical tools

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World Journal of Critical Care Medicine 2022年第1期11卷 1-21页

作者： Juan Carlos Ruiz-Rodriguez Erika P Plata-Menchaca Luis Chiscano-Camón adolfo Ruiz-Sanmartin Marcos Pérez-Carrasco Clara Palmada Vicent Ribas Mónica Martínez-Gallo Manuel Hernández-González Juan J Gonzalez-lopez Nieves Larrosa Ricard Ferrer Intensive Care department Vall d’Hebron Hospital UniversitariVall d’Hebron Barcelona Hospital CampusBarcelona 08035Spain Shock Organ Dysfunction and Resuscitation Research GroupVall d’Hebron Institut de Recerca(VHIR)Vall d’Hebron Barcelona Hospital CampusBarcelona 08035Spain departament de Medicina Universitat Autònoma de BarcelonaBellaterra 08193Spain department of Intensive Care Hospital Clínic de BarcelonaBarcelona 08036Spain Data Analytics in Medicine Digital Health UnitEurecatCentre Tecnològic de CatalunyaBarcelona 08005Spain Immunology Division Vall d’Hebron Hospital UniversitariVall d’Hebron Barcelona Hospital CampusBarcelona 08035Spain Diagnostic Immunology Research Group Vall d’Hebron Institut de Recerca(VHIR)Vall d’Hebron Barcelona Hospital CampusBarcelona 08035Spain department of Cell Biology Physiology and ImmunologyUniversitat Autònoma de BarcelonaBellaterra 08193Spain department of Clinical Microbiology Vall d’Hebron Hospital UniversitariVall d’Hebron Barcelona Hospital CampusBarcelona 08035Spain department of Microbiology and Genetics Universitat Autònoma de BarcelonaBellaterra 08193Spain

Sepsis is a heterogeneous disease with variable clinical course and several clinical *** it is associated with an increased risk of death,patients with this condition are candidates for receipt of a very well-structured and protocolized *** patients should receive the fundamental pillars of sepsis management,which are infection control,initial resuscitation,and multiorgan ***,specific subgroups of patients may benefit from a personalized approach with interventions targeted towards specific pathophysiological ***,we will review the framework for identifying subpopulations of patients with sepsis,septic shock,and multiorgan dysfunction who may benefit from specific *** of these approaches are still in the early stages of research,while others are already in routine use in clinical practice,but together will help in the effective generation and safe implementation of precision medicine in sepsis.

关键词： Sepsis Septic shock Organ dysfunction Precision medicine Biomarkers Phenotype Endotype

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