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World Journal of Pediatrics 2015年第3期11卷 272-275页

作者： ernesto martin-nunez Elizabeth Cordoba-Lanus Hilaria Gonzalez-Acosta Aniana Oliet Elvira Izquierdo Felix Claverie-martin Unidad de Investigacion Hospital Nuestra Senora de CandelariaSanta Cruz de TenerifeSpain Servicio de Nefrologia Hospital Severo OchoaMadridSpain Nefrologia Infantil Hospital General Gregorio MaranonMadridSpain

Background:Familial hypomagnesemia with hypercalciuria and nephrocalcinosis(FHHNC)is an autosomal recessive tubular disease caused by mutations in the CLDN16 or CLDN19 *** studies using microsatellite markers flanking the CLDN19 locus estimated that p.G20D(c.59G>A),a recurrent mutation in Spanish families,is a founder *** the present study,we assessed the haplotype of Spanish patients using single nucleotide polymorphisms(SNPs).Methods:Twenty-seven FHHNC patients were included in this *** analyzed four SNPs located in CLDN19 introns 3 and 4 by polymerase chain reaction amplification and DNA ***:Three new patients with homozygous p.G20D were *** SNP genotyping analysis showed that alleles carrying this mutation shared a common SNP ***:Our findings suggest the existence of a founder effect responsible for FHHNC in our *** for the presence of mutation p.G20D should be the first genetic screening in Spanish patients.

关键词： chronic kidney disease founder effect gene mutation nephrocalcinosis

Daptomycin,Methicillin Resistant Staphylococcus hominis Catheter-Related Bacteraemia in a Hemodialysis Patient

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Open Journal of Medical Microbiology 2013年第1期3卷 53-55页

作者： Manuel Macia-Heras Javier Donate-Correa Julia Alcoba-Florez Gerardo Pulido-Reyes ernesto martin-nunez Joel martin-Padron Sebastian Mendez-Alvarez Research Unit University Hospital Nuestra Senora de CandelariaSanta Cruz de TenerifeSpain Nephrology Service University Hospital Nuestra Senora de CandelariaSanta Cruz de TenerifeSpain Microbiology Service University Hospital Nuestra Senora de CandelariaSanta Cruz de TenerifeSpain Department of Microbiology and Cell Biology University of La LagunaTenerifeSpain

We report a case of a haemodialysis patient that presented a catheter-related bacteraemia caused by a Coagulase negative Staphylococcus. With the utilization of molecular biology techniques the bacterial isolate recovered from catheter was surprisingly identified as S. hominis by sequencing of the 16S ribosomal gene. The S. hominis isolate, which is not often associated with infections in dialysis patients, was resistant to methicillin, being mecA positive, and to daptomycin. The patient was successfully treated with vancomycin together with the catheter retirement.

关键词： Bacteraemia Catheter Daptomycin Methicillin Staphylococcus hominis

Klotho in cardiovascular disease:Current and future perspectives

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World Journal of Biological Chemistry 2015年第4期6卷 351-357页

作者： Javier Donate-Correa ernesto martin-nunez Carmen Mora-Fernandez Mercedes Muros-de-Fuentes Nayra Perez-Delgado Juan F Navarro-Gonzalez Research Unit University Hospital Nuestra Senora de Candelaria Clinical Biochemistry Service University Hospital Nuestra Senora de Candelaria Nephrology Service University Hospital Nuestr Senora de Candelaria

Protein Klotho,beyond its role as a regulator of the phosphatemia,is also involved in the maintaining of the cardiovascular health,being associated its alterations with the development of cardiovascular damage and increased morbi-mortality. For all this,nowadays Klotho is the subject of a thorough research which is focused on uncover its intimate mechanisms of action,and in analyzing the utility of its modulation as a potential strategy with clinical applicability. Molecular mechanisms of Klotho are not well understood but an emerging research area links Klotho deficiency with vascular pathology. Changes in this protein have been associated with cardiovascular-related complications like inflammation,vascular calcification,and endothelial dysfunction. All this is particularly relevant if considering the recent discovery of Klotho expression in vascular tissue.

关键词： Klotho Cardiovascular disease Chronic kidney disea

Acquired sensorineural hearing loss,oxidative stress,and microRNAs

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Neural Regeneration Research 2025年第9期20卷 2513-2519页

作者： Desmond A.nunez Ru C.Guo Division of Otolaryngology–Head&Neck Surgery Department of SurgeryFaculty of MedicineThe University of British ColumbiaVancouverBCCanada Division of Otolaryngology–Head&Neck Surgery Gordon&Leslie Diamond Health Care CentreVancouver General HospitalVancouverBCCanada Faculty of Medicine–The University of British Columbia VancouverBCCanada

Hearing loss is the third leading cause of human ***-related hearing loss,one type of acquired sensorineural hearing loss,is largely responsible for this escalating global health ***-induced,ototoxic,and idiopathic sudden sensorineural are other less common types of acquired hearing *** etiology of these conditions is complex and multi-fa ctorial involving an interplay of genetic and environmental *** stress has recently been proposed as a likely linking cause in most types of acquired sensorineural hearing *** non-coding RNA sequences known as microRNAs(miRNAs)have increasingly been shown to play a role in cellular hypoxia and oxidative stress responses including promoting an apoptotic *** hair cell death is a central histopathological finding in sensorineural hearing *** these cells do not regenerate in humans,it underlies the irreversibility of human age-related hearing *** EMBASE,Ovid MEDLINE,Web of Science Core Collection,and *** databases over the period August 1,2018 to July 31,2023 were searched with"hearing loss,""hypoxamiRs,""hypoxia,""microRNAs,""ischemia,"and"oxidative stress"text words for English language primary study publications or registered clinical *** red clinical trials known to the senior author we re also assessed.A total of 222studies were thus *** excluding duplicates,editorials,retra ctions,secondary research studies,and non-English language articles,39 primary studies and clinical trials underwent full-text *** resulted in 11 animal,in vitro,and/or human subject journal articles and 8 registered clinical trial database entries which form the basis of this narrative *** miR-34a and miR-29b levels increase with age in *** miRNAs were demonstrated in human neuroblastoma and murine cochlear cell lines to target Sirtuin 1/peroxisome proliferato r-activated receptor gamma coactivator-1-alpha(SIRT1/P GC-1α),SIRT

关键词： hearing loss hypoxia microRNAs oxidative stress sensorineural

From single to combinatorial therapies in spinal cord injuries for structural and functional restoration

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Neural Regeneration Research 2025年第3期20卷 660-670页

作者： ernesto Doncel-Pérez Gabriel Guízar-Sahagún Israel Grijalva-Otero Hospital Nacional de Parapléjicos de Toledo Servicio de Salud de Castilla La Mancha(SESCAM)ToledoSpain Medical Research Unit for Neurological Diseases UMAE Hospital de EspecialidadesCentro Médico Nacional Siglo XXIIMSSCiudad de MéxicoMéxico

Spinal cord injury results in paralysis, sensory disturbances, sphincter dysfunction, and multiple systemic secondary conditions, most arising from autonomic dysregulation. All this produces profound negative psychosocial implications for affected people, their families, and their communities;the financial costs can be challenging for their families and health institutions. Treatments aimed at restoring the spinal cord after spinal cord injury, which have been tested in animal models or clinical trials, generally seek to counteract one or more of the secondary mechanisms of injury to limit the extent of the initial damage. Most published works on structural/functional restoration in acute and chronic spinal cord injury stages use a single type of treatment: a drug or trophic factor, transplant of a cell type, and implantation of a biomaterial. Despite the significant benefits reported in animal models, when translating these successful therapeutic strategies to humans, the result in clinical trials has been considered of little relevance because the improvement, when present, is usually insufficient. Until now, most studies designed to promote neuroprotection or regeneration at different stages after spinal cord injury have used single treatments. Considering the occurrence of various secondary mechanisms of injury in the acute and sub-acute phases of spinal cord injury, it is reasonable to speculate that more than one therapeutic agent could be required to promote structural and functional restoration of the damaged spinal cord. Treatments that combine several therapeutic agents, targeting different mechanisms of injury, which, when used as a single therapy, have shown some benefits, allow us to assume that they will have synergistic beneficial effects. Thus, this narrative review article aims to summarize current trends in the use of strategies that combine therapeutic agents administered simultaneously or sequentially, seeking structural and functional restoratio

关键词： neural regeneration neuroprotection spinal cord injury repair spinal cord injury treatments structural restoration of spinal cord injury

Emerging insights into the function of very long chain fatty acids at cerebellar synapses

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Neural Regeneration Research 2025年第6期20卷 1709-1710页

作者： martin-Paul Agbaga Mohiuddin Ahmad Department of Cell Biology and Ophthalmology University of Oklahoma Health Sciences Centerand Dean McGee Eye InstituteOklahoma CityOKUSA

Very long chain-saturated and-polyunsaturated fatty acids(VLC-SFA and VLC-PUFA, respectively) are a functionally important class of fatty acids containing 28 carbons or more in their acyl chain. They are synthesized by the elongation of very long fatty acids-4(ELOVL4) enzyme, expressed mainly in the brain, retina, skin, testes, and meibomian gland, where these fatty acids are found(Agbaga et al., 2008). Further, these organs exhibit tissuespecific VLC-PUFA and VLC-SFA biosynthesis and incorporation into complex lipids for specific functions. In the brain, skin, and Meibomian glands, the ELOVL4 mainly makes VLC-SFA, which are incorporated into complex sphingolipids. In the retina, the ELOVL4 makes VLC-PUFA that are incorporated into phosphatidylcholine, that are critical for visual function, while in testes and sperm, the VLC-PUFA are incorporated into sphingolipids that are critical for fertility(Yeboah et al., 2021).

关键词： functions function insight

Alternative Strategy for Development of Dielectric Calcium Copper Titanate‑Based Electrolytes for Low‑Temperature Solid Oxide Fuel Cells

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Nano-Micro Letters 2025年第1期17卷 310-332页

作者： Sajid Rauf Muhammad Bilal Hanif Zuhra Tayyab Matej Veis MAKYousaf Shah Naveed Mushtaq Dmitry Medvedev Yibin Tian Chen Xia martin Motola Bin Zhu College of Mechatronics and Control Engineering Shenzhen UniversityShenzhen 518060People’s Republic of China Department of Inorganic Chemistry Faculty of Natural SciencesComenius University BratislavaIlkovicova684215 BratislavaSlovakia Energy Storage Joint Research Center School of Energy and EnvironmentSoutheast UniversityNanjing 210096People’s Republic of China Hydrogen Energy Laboratory Ural Federal University620002 EkaterinburgRussia Laboratory of Electrochemical Devices Based on Solid Oxide Proton Electrolytes Institute of High Temperature Electrochemistry620066 EkaterinburgRussia School of Microelectronics Hubei UniversityWuhan 430062People’s Republic of China

The development of low-temperature solid oxide fuel cells(LT-SOFCs)is of significant importance for realizing the widespread application of *** has stimulated a substantial materials research effort in developing high oxide-ion conductivity in the electrolyte layer of *** this context,for the first time,a dielectric material,CaCu_(3)Ti_(4)O_(12)(CCTO)is designed for LT-SOFCs electrolyte application in this *** individual CCTO and its heterostructure materials with a p-type Ni_(0.8)Co_(0.15)Al_(0.05)LiO_(2−δ)(NCAL)semiconductor are evaluated as alternative electrolytes in LT-SOFC at 450–550℃.The single cell with the individual CCTO electrolyte exhibits a power output of approximately 263 mW cm^(-2) and an open-circuit voltage(OCV)of 0.95 V at 550℃,while the cell with the CCTO–NCAL heterostructure electrolyte capably delivers an improved power output of approximately 605 mW cm^(-2) along with a higher OCV over 1.0 V,which indicates the introduction of high hole-conducting NCAL into the CCTO could enhance the cell performance rather than inducing any potential short-circuiting *** is found that these promising outcomes are due to the interplay of the dielectric material,its structure,and overall properties that led to improve electrochemical mechanism in CCTO–***,density functional theory calculations provide the detailed information about the electronic and structural properties of the CCTO and NCAL and their heterostructure CCTO–*** study thus provides a new approach for developing new advanced electrolytes for LT-SOFCs.

关键词： LT-SOFCs Dielectric CaCu_(3)Ti_(4)O_(12) Semiconductor Ni_(0.8)Co_(0.15)Al_(0.05)LiO_(2−δ) Ionic conductivity Heterostructure electrolyte

Movement analysis in the diagnosis and management of Parkinson’s disease

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Neural Regeneration Research 2025年第2期20卷 485-486页

作者： Johannes Burtscher Nicolas Bourdillon Jules MJanssen Daalen Aurélien Patoz Julien FBally martin Kopp Davide Malatesta Bastiaan RBloem Institute of Sport Sciences University of LausanneLausanneSwitzerland Radboud University Medical Center Department of NeurologyDonders Institute for BrainCognition and BehaviorCenter of Expertise for Parkinson and Movement DisordersNijmegenThe Netherlands Radboud University Medical Center Department of Medical BioSciencesNijmegenThe Netherlands Research and Development Department Volodalen Swiss Sport LabAigleSwitzerland Service of Neurology Department of Clinical NeurosciencesLausanne University Hospital(CHUV)and University of Lausanne(UNIL)LausanneSwitzerland Department of Sport Science University of InnsbruckInnsbruckAustria

Challenges in the diagnosis and treatment of Parkinson’s disease:Parkinson’s disease(PD)is an increasingly prevalent neurodegenerative disease,at first sight primarily characterized by motor symptoms,although non-motor symptoms also constitute a major part of the overall ***,this disease cannot be diagnosed reliably until a large part of the vulnerable dopaminergic neurons has been irretrievably lost,and the disease progresses *** biological criteria for PD have been proposed recently and might eventually improve early diagnosis,but they require further validation,and their use will initially be restricted to a research environment(Darweesh et al.,2024).

关键词： diagnosis Clinical eventually

Self-Organized Growth of Complex Nanotube Patterns on Crystal Surfaces

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Nano Research 2009年第10期2卷 743-754页

作者： ernesto Joselevich Department of Materials and Interfaces Weizmann Institute of ScienceRehovot 76100Israel

The organization of carbon nanotubes into well-dened straight or curved geometries and arrays on surfaces is a critical prerequisite for their integration into nanocircuits and a variety of functional *** review the recent development of a new approach to carbon nanotube organization based on self-organized growth directed by well-defined crystal surfaces,or“nanotube epitaxy”.We identify three different modes of surface-directed growth,namely by atomic rows,atomic steps,and *** emphasis is given here to the combinations of such surface-directed growth with external forces-like those exerted by an electriceld or gas o-wfor the creation of well-de-ned complex geometries,including crossbar architectures,serpentines,and coils.

关键词： Nanotubes self-organization surface science nanostructures nanofabrication self-assembly

Study of geometric parameters for the design of short intakes with fan modelling

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Chinese Journal of Aeronautics 2022年第11期35卷 18-32页

作者： Andrea MAGRINI ernesto BENINI Dipartimento di Ingegneria Industriale Universitá degli Studi di PadovaPadova 35131Italy

The flow over a short intake is characterised by a strong interaction with the fan, that can only be captured when the rotor blades are modelled in the numerical simulations. In this paper, we use a coupled methodology to derive indications about relevant geometric variables affecting the high-incidence operation of an ultra-high bypass ratio turbofan intake with a length-to-diameter ratio of 0.35. By reproducing the effect of the fan through a body force model, we carry out a parametric study of the influence of the contraction ratio and the scarf angle at take-off conditions for a grid of 28 different three-dimensional shapes. The analysis of the selected performance metrics distributions at three angles of attack of 16., 24., and 28. reveals that a contraction ratio higher than 1.20 is needed to avoid separation at high incidence. While for an attached inlet the best performance is found with a moderate scarf angle, in presence of a developed separation the distortion level reduces as the scarf decreases up to negative values. We discuss the correspondence between the distortion indexes and the flow field, highlighting the origin of the detachment for the different geometries, according to the operating condition, and analysing the fan operation in the most distorted case. Finally, we assess the influence of modelling the rotor in the simulations, showing that its suppression effect on the separation at a given incidence depends on the intake geometric features.

关键词： Body force method Coupled methods Fan/intake interaction Nacelle aerodynamics Short intake UHBPR