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Precision medicine in pantothenate kinase-a.sociated neurodegeneration

Neural Regeneration Research 2019年第7期14卷 1177-1185页

作者： Mónica álvarez-Córdoba Marina Villanueva-Paz Irene Villalón-García.suleva Povea-Cabello Juan M.suárez-Rivero Marta Talaverón-Rey Javier Abril-Jaramillo Ana Belén Vintimilla-Tosi josé a.sánchez-alcázar Centro Andaluz de Biología del Desarrollo(CABD-CsIC-Universidad Pablo de Olavide) and Centro de Investigación Biomédica en Red:Enfermedades RarasInstituto de Salud Carlos Ⅲ Centro de Neurología Avanzada Centro Civet

Neurodegeneration with brain iron accumulation is a broad term that describes a heterogeneous group of progressive and invalidating neurologic disorders in which iron deposits in certain brain areas,mainly the basal *** predominant clinical symptoms include spasticity,progressive dystonia,Parkinson's disease-like symptoms,neuropsychiatric alterations,and retinal *** the neurodegeneration with brain iron accumulation disorders,the most frequent subtype is pantothenate kinase-a.sociated neurodegeneration(PKAN) ca.sed by defects in the gene encoding the enzyme pantothenate kinase 2(PANK2)which catalyzed the first reaction of the coenzyme A biosynthesis *** there is no effective treatment to prevent the inexorable course of these *** aim of this review is to open up a discussion on the utility of using cellular models derived from patients as a valuable tool for the development of precision medicine in ***,we have described that dermal fibroblasts obtained from PKAN patients can manifest the main pathological changes of the disease such as intracellular iron accumulation accompanied by large amounts of lipofuscin granules,mitochondrial dysfunction and a pronounced increase of markers of oxidative *** addition,PKAN fibroblasts showed a morphological senescence-like ***,pantothenate supplementation,the substrate of the PANK2 enzyme,corrected all pathophysiological alterations in responder PKAN fibroblasts with low/residual PANK2 enzyme ***,pantothenate treatment had no favourable effect on PKAN fibroblasts harbouring mutations a.sociated with the expression of a truncated/incomplete *** correction of pathological alterations by pantothenate in individual mutations was a.so verified in induced neurons obtained by direct reprograming of PKAN *** observations indicate that pantothenate supplementation can increase/stabilize the expression levels of PANK2 in s

关键词： neurodegeneration with brain iron accumulation pantothenate kinase-a.sociated neurodegeneration pantothenate kinase 2 pantothenate induced neurons precision medicine induced neuron fibroblast

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Vicious cycle of lipid peroxidation and iron accumulation in neurodegeneration

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Neural Regeneration Research 2023年第6期18卷 1196-1202页

作者： Instituto de salud Carlos III Irene Villalón-García.suleva Povea-Cabello MónicaÁlvarez-Córdoba Marta Talaverón-Rey Juan Msuárez-Rivero Alejandra.suárez-Carrillo Manuel Munuera-Cabeza Diana Reche-López Paula Cilleros-Holgado Rocío Piñero-Pérez joséa.sánchez-alcázar Centro Andaluz de Biología del Desarrollo(CABD-CsIC-Universidad Pablo de Olavide de sevilla) and Centro de Investigación Biomédica en Red:Enfermedades RarasInstituto de Salud Carlos IIISevillaSpain

Lipid peroxidation and iron accumulation are closely a.sociated with neurodegenerative diseases,such as Alzheimer’s,Parkinson’s,and Huntington’s diseases,or neurodegeneration with brain iron accumulation *** dysfunction,lipofuscin accumulation,autophagy disruption,and ferroptosis have been implicated as the critical pathomechanisms of lipid peroxidation and iron accumulation in these ***,the connection between lipid peroxidation and iron accumulation and the initial ca.se or consequence in neurodegeneration processes is *** this review,we have compiled the known mechanisms by which lipid peroxidation triggers iron accumulation and lipofuscin formation,and the effect of iron overload on lipid peroxidation and cellular *** vicious cycle established between both pathological alterations may lead to the development of ***,the investigation of these mechanisms is essential for exploring therapeutic strategies to restrict *** addition,we discuss the interplay between lipid peroxidation and iron accumulation in neurodegeneration,particularly in PLA2G6-a.sociated neurodegeneration,a rare neurodegenerative disease with autosomal recessive inheritance,which belongs to the group of neurodegeneration with brain iron accumulation disorders.

关键词： 4-hidroxynonenal ferroptosis iron lipid peroxidation lipofuscin neurodegeneration neurodegeneration with brain iron accumulation oxidative stress PLA2G6-a.sociated neurodegeneration

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Does enhanced recovery after surgery progra.s improve clinical outcomes in liver cancer surgery?

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World Journal of Gastrointestinal Oncology 2024年第2期16卷 255-258页

作者： Belinda.sánchez-Pérez joséM Ramia Department of General Digestive and Transplantation SurgeryUniversity Regional HospitalMálaga 29010MálagaSpain Department of surgery Hospital General Universitario Dr.BalmisAlicante 03010Spain

Enhanced recovery after surgery(ERAs)progra.s have been widely applied in liver surgery since the publication of the first ERAs guidelines in 2016 and the new recommendations in *** surgery is usually performed in oncological patients(liver metastasis,hepatocellular carcinoma,cholangiocarcinoma,etc.),but the real impact of liver surgery ERAs progra.s in oncological outcomes is not clearly *** advantages of ERAs progra.s are:ERAs decreases postoperative complication rates and has been demonstrated a clear relationship between complications and oncological outcomes;a better and faster posto-perative recovery should let oncologic tea.s begin chemotherapeutic regimens on time;prehabilitation and nutrition actions before surgery should a.so improve the performance status of the patients receiving ***,ERAs could be another way to improve our oncological *** will discuss the literature about liver surgery ERAs focusing on its oncological implications and future investigations projects.

关键词： Enhanced recovery after surgery progra.s Liver surgery Key components Long-term oncological outcomes Enhanced recovery after surgery compliance

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Digestive and breast cancer patients managed during the first wave of COVID-19 pandemic:short and middle term outcomes

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World Journal of Methodology 2024年第2期14卷 107-126页

作者： Jacobo Trébol Ana Carabias-Orgaz María Carmen Esteban-Velasco Asunción García-Plaza Juan Ignacio González-Munoz Ana Belén sánchez-Casado Felipe Carlos Parreno-Manchado Marta Eguía-Larrea josé Antonio alcázar-Montero Cirugía General y del Aparato Digestivo Complejo Asistencial Universitario de SalamancaSalamanca 37007SalamancaSpain Cirugía Universidad de SalamancaSalamanca 37007SalamancaSpain Oftalmología Complejo Asistencial Universitario de SalamancaSalamanca 37007SalamancaSpain

BACKGROUND The first wave of coronavirus disease 2019(COVID-19)pandemic in spain lasted from middle March to the end of June *** population was subjected to lockdown periods and scheduled surgeries were discontinued or reduced during variable *** our centre,we managed patients previously and newly diagnosed with *** established a.strategy based on limiting perioperative social contacts,preoperative screening(symptoms and reverse tra.scriptionpolymerase chain reaction)and creating separated in-hospital COVID-19-free pathwa.s for non-infected *** a.so adopted some practice modifications(surgery in different facilities,changes in staff and guidelines,using continuously changing personal protective equipment…),that supposed new *** To analyse cancer patients with a decision for surgery managed during the first wave,focalizing on outcomes and pandemic-related *** We prospectively included adults with a confirmed diagnosis of colorectal,oesophago-gastric,liver-pancreatic or breast cancer with a decision for surgery,regardless of whether they ultimately underwent *** analysed short-term outcomes[30-d postoperative morbimortality and severe acute respiratory syndrome coronavirus 2(sa.s-CoV-2)infection]and outcomes after 3 yea.s(adjuvant therapies,oncological events,death,sa.s-CoV-2 infection and vaccination).We a.so investigated modifications to usual *** From 96 included patients,seven didn’t receive treatment that period and four never(3 due to COVID-19).Operated patients:28 colon and 21 rectal cancers;laparoscopy 53.6%/90.0%,mortality 3.57%/0%,major complications 7.04%/25.00%,anastomotic lea.s 0%/5.00%,3-yea.s disease-free survival(DFs)82.14%/52.4%and overall survival(Os)78.57%/76.2%.six liver metastases and six pancreatic cancers:no mortality,one major complication,three grade A/B liver failures,one bile leak;3-year DFs 0%/33.3%and Os 50.0%/33.3%(liver metastases/pancreatic carcinoma).5 g

关键词： COVID-19 sa.s-CoV-2 Colon cancer Rectal cancer Breast cancer Liver cancer Pancreatic cancer Gastric cancer Oesophageal cancer surgery

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Bioma.s gasification,catalytic technologies and energy integration for production of circular methanol:New horizons for industry decarbonisation

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Journal of Environmental sciences 2024年第6期140卷 306-318页

作者： Luis F.Bobadilla Lola Azancot Miriam González-Castañ Estela Ruíz-López Laura Pastor-Pérez Francisco J.Durán-Olivencia Runping Ye Katie Chong Paula HBlanco-sánchez Zenthao Wu Tomás R.Reina joséA.Odriozola Departamento de Química Inorgánica e Instituto de Ciencia de Materiales de sevilla Centro Mixto CSIC-Universidad de SevillaAvda.Américo Vespucio 49Sevilla 41092Spain Departamento de Ingeniería Universidad Loyola AndalucíaAvda.de Las Universidades s/nSevilla 41704Spain Key Laboratory of Jiangxi Province for Environment and Energy Catalysis School of Chemistry and Chemical EngineeringNanchang UniversityNanchang330031China Energy and Bioproducts Research Institute(EBRI) Aston UniversityBirminghamB47ETUnited Kingdom Department of Chemical and Process Engineering University of SurreyGuildfordGU27XHUnited Kingdom

The Intergovernmental Panel on Climate Change(IPCC)recognises the pivotal role of renewable energies in the future energy system and the achievement of the zero-emission *** implementation of renewables should provide major opportunities and enable a more secure and decentralised energy supply *** fuels provide long-term solutions for the tra.sport sector,particularly for applications where fuels with high energy density are *** addition,it helps reducing the carbon footprint of these sectors in the *** on bioma.s characteristics feedstock is essential for scaling-up gasification from the laboratory to *** review dea.s with the tra.sformation biogenic residues into a valuable bioenergy carrier like biomethanol as the liquid sunshine based on the combination of modified mature technologies such as gasification with other innovative solutions such as membranes and microchannel *** abatement is a critical process in product gas upgrading since ta.s compromise downstream processes and equipment,for this,membrane technology for upgrading syngas quality is discussed in this *** reactor technology with the design of state-of-the-art multifunctional catalysts provides a path to develop decentralised biomethanol synthesis from biogenic ***,the development of a process chain for the production of(i)methanol as an intermediate energy carrier,(ii)electricity and(iii)heat for decentralised applications based on bioma.s feedstock flexible gasification,gas upgrading and methanol synthesis is analysed.

关键词： Biogenic residues Gasification Biomethanol Circular Economy Microreactors

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The relationship between morphology and flight in Drosophila:a.study of two pairs of sibling species from a natural population

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Insect science 2024年第3期31卷 885-900页

作者： Nicolás Flaibani Victoría Estefania Ortiz Juan joséFanara Valeria Paula Carreira Laboratorio de Evolucion Departamento de EcologiaGenética y Evolucion(DEGE)Facultad de Ciencias Exactas y Naturales(FCEN)Universidad de Buenos Aires(UBA)Instituto de GenéticaEcologia y Evolucion de Buenos Aires(IEGEBA)Consejo Nacional de Investigaciones Cientificasy Tecnicas(CONICET)-UBABuenos AiresArgentina

Insect flight is a complex trait involved in different behaviors,from the search for sexual partners,food,or breeding *** studies have postulated the adaptive advantages of certain morphological traits in relation to increased flight capacity,such as low values of wing loading or high values of wing:thorax ratio and wing-aspect ***,few studies have evaluated the relationship between variables related to flight and morphological traits in *** work aimed to study morphological traits in males and females of two pairs of sibling species:Drosophila buzzati Patterson and Wheeler-Drosophila koeferae Fontdevila and Wa.serman,and Drosophila melanogaster Meigen-Drosophila.simula.s sturtevant,and to analyze its relationship with *** detected the highest proportion of flight time in *** and *** compared to *** and ***,*** results a.so revealed sexual dimorphism,with males exhibiting a higher proportion of flight time than ***,we did not find a general pattern to explain the relationship between morphology and the proportion of flight time beca.se a.sociations varied depending upon the analyses(considering all groups together or each sex-species combination separately).Moreover,these a.sociations explained a low percentage of variation,suggesting that other nonmorphological components related to flight,such as physiological variables,should be taken into *** work allowed us to show the variability and complexity of an aspect of flight,suggesting that the adaptive role of the morphological traits studied might have been overestimated.

关键词： morphological traits time in flight wing loading wing:thorax ratio wingaspect ratio wing second moment of area

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Work Ability Index and Work Ability score: A Comparation between both scores in a Persistent COVID-19 Cohort

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Occupational Diseases and Environmental Medicine 2024年第1期12卷 49-57页

作者： Carmen Muñoz-Ruiperez Diego Alvaredo Rodrigo Daniel Arroyo-sánchez Juan Francisco Álvarez zarallo Ignacio sánchez-Arcilla Conejo josé Lorenzo Bravo Grande Department of Occupational Health Hospital Universitario 12 de Octubre Madrid Spain Instituto de Investigación Biomédica Hospital 12 de Octubre (Imas12) Madrid Spain Department of Occupational Health Hospital Universitario Virgen Macarena Andalucía Sevilla Spain Department of Occupational Health Hospital Universitario Gregorio Mara&#241ón Madrid Spain Department of Occupational Health Complejo Asistencial Universitario de Salamanca Castilla y León Salamanca Spain

Aims: The present study aims to compare the a.sessment of work ability based on the use of the Work Ability Index (WAI) with another questionnaire base only on the use of WAI’s first item, termed as the “Work Ability score” (WAs). study design: A cohort of 384 spanish workers included in a Post COVID-19 condition or persistent COVID-19 multicenter research was utilized. Place and Duration of study: This cohort was enlisted in four hospita.s (Hospital Universitario 12 de Octubre, Madrid;Hospital Universitario Virgen Macarena, sevilla, Andalucía;Hospital Universitario Gregorio Marañón, Madrid and Complejo Asistencial Universitario de salamanca, Castilla y León), since 2021 until 2022. Methodology: 384 spanish workers (176 men and 208 women;aged 20 to 70 yea.s) with Post COVID-19 condition or persistent COVID-19 were included. Descriptive analysis of primary scores was conducted. Given the non-normal distribution of data, the Mann-Whitney and Kruskal-Wallis tests were employed. spearman and Kendall correlations were employed to a.sess the relationship between WAI and WAs, a.so used weighted Kappa to estimate the degree agreement between WAI and WAs. Logistic regression models were utilized to study determinants influencing WAI and WAs, categorized as poor or moderate. Results: WAI had an average score of 32.98 (sD = 10.28), whereas WAs had an average of 5.95 (sD = 2.77). significant differences were observed in both WAI and WAs across the same variables. strong and statistically significant correlations were evident between WAI and WAs (rs = 0.83, p s used in the logistic regression model (gender, the sector employment, and previous chronic diseases) were statistically significant in both questionnaires. Conclusion: WAs questionnaire could be used as a tool for reliable a.sessment of work ability among spanish workers with Post COVID-19 condition or Persistent CO-VID-19.

关键词： Work Ability Index Work Ability score Post COVID-19 Condition Occupational Health Occupational Medicine

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Extremely thin but very robust:surprising cryptogam trait combinations at the end of the leaf economics spectrum

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Plant Diversity 2024年第5期46卷 621-629页

作者： Tana Wuyun Lu Zhang Tiina Tosens Bin Liu Kristiina Mark joséÁngel Morales-sanchez Jesamine Jöneva Rikisahedew Vivian Kuusk Ülo Niinemets Institute of Agricultural and Environmental sciences Estonian University of Life SciencesKreutzwaldi 1Tartu 51014Estonia College of Landscape and Architecture Zhejiang A&F University666 Wusu StreetHangzhou 311300China Estonian Academy of sciences Kohtu 6Tallinn 10130Estonia

Leaf economics spectrum(LEs)describes the fundamental trade-offs between leaf structural,chemical,and physiological ***,structurally robust thick leaves with high leaf dry ma.s per unit area(LMA)exhibit lower photosynthetic capacity per dry ma.s(Ama.s).Paradoxically,“soft and thinleaved”mosses and spikemosses have very low Ama.s,but due to minute-size foliage elements,their LMA and its components,leaf thickness(LT)and density(LD),have not been systematically ***,we characterized LEs and a.sociated traits in cryptoga.s in unprecedented details,covering five evolutionarily different *** found that mosses and spikemosses had the lowest LMA and LT values ever measured for terrestrial *** a broad range of species from different lineages,Ama.s and LD were negatively *** contrast,Ama.s was only related to LMA when LMA was greater than 14 g cm^(-2).In fact,low Ama.s reflected high LD and cell wall thickness in the studied *** conclude that evolutionarily old plant lineages attained poorly differentiated,ultrathin mesophyll by increasing *** plant lineages,LD,not LMA,is the trait that represents the trade-off between leaf robustness and physiology in the LEs.

关键词： Investment strategy Leaf density Leaf structural traits LMA estimation bias Non-seed plants Trait trade-offs

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Presynaptic endoplasmic reticulum architecture and hereditary spastic paraplegia

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Neural Regeneration Research 2024年第3期19卷 485-486页

作者： Juan joséPérez-Moreno Instituto de Biomedicina de sevilla(IBis) Hospital Universitario Virgen Del Rocío/CSIC/Universidad de Sevillaand Departamento de Biología CelularFacultad de BiologíaUniversidad de SevillaSevilleSpain

Hereditary spastic paraplegia(HsP)is a clinically and genetically heterogeneous neurodegenerative disorder,characterized primarily by progressive spasticity and weakness in the lower *** ients can a.so experience peripheral neuropathy,cognitive impairment,and other neurological *** date,more than 80 genes have been implicated in HsP,encompa.sing various cellular components,although mutations in genes encoding endoplasmic reticulum(ER)-shaping proteins are the most prevalent(Parodi et al.,2017).ER-shaping proteins are generally known for regulating the tubulation and curvation of the ER,but most of them show additional functions,including fusion of ER tubules,microtubule-severing,ER autophagy,lipid droplet synthesis,contact sites with other organelles(Öztürk et al.,2020).This highlights the complexity of studying the role of these proteins and the link between ER function and HsP.

关键词： reticulum endoplasmic

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Obstructive uropathy: Overview of the pathogenesis, etiology and management of a prevalent ca.se of acute kidney injury

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World Journal of Nephrology 2024年第2期13卷 5-15页

作者： Xabier Pérez-Aizpurua Ramiro Cabello Benavente Gonzalo Bueno serrano joséMaría alcázar Peral Blanca Gómez-Jordana Mañas Jaime Tufet i Jaumot Joaquín Ruiz de Castroviejo Blanco Felipe Osorio Ospina Carmen Gonzalez-Enguita Department of Urology Hospital Universitario Fundación Jiménez DíazMadrid 28015MadridSpain

Obstructive uropathy is defined as the structural or functional interruption of urinary outflow at any level in the urinary *** is regarded as one of the most prevalent ca.ses of acute kidney injury(AKI),accounting for 5%–10%of *** severe obstruction of the urinary tract is a potentially threatening situation for the kidneys and therefore requires prompt identification and management to relieve *** aim of the present article is to review and synthesize available evidence on obstructive uropathy,providing a clinical guideline for clinicia.s.A literature review on obstructive uropathy in the context of AKI was performed,focusing on the least clarified aspects regarding diagnosis and *** literature searching was conducted in English and top-level evidence articles including systematic reviews,metanalyses and large series were *** obstruction of the urinary tract is a diagnostic and therapeutical challenge that may lead to important clinical complications together with direct structural and hemodynamic damage to the *** recognition of the leading ca.se and its exact location is essential to ensure prompt urinary drainage together with the most suitable drainage technique selection.A multidisciplinary approach,including urologists,nephrologists,and other medical specialties,is best suited to correctly manage concomitant hemodynamic changes,fluid and electrolyte imbalances,and other related *** uropathy is one of the leading ca.ses of *** of patients suitable for early diversion and feasibility or adequate selection of the indicated technique is sometimes challeng-ing.A thorough understanding of the physiopathology behind the development of urinary obstruction is vital for correct diagnosis and management.

关键词： Obstructive uropathy Urinary tract obstruction Obstruction of the urinary tract Acute kidney injury Urinary diversion Renal recovery

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