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Alexander disease:the road ahead

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Neural Regeneration Research 2023年第10期18卷 2156-2160页

作者： María A.pajares Elena Hernández-Gerez Milos pekny Dolores p.rez-Sala Department of Structural and Chemical Biology Centro de Investigaciones Biológicas Margarita SalasMadridSpain Laboratory of Astrocyte Biology and CNS Regeneration Center for Brain RepairDepartment of Clinical NeuroscienceInstitute of Neuroscience and PhysiologySahlgrenska Academy at the University of GothenburgGothenburgSweden University of Newcastle NewcastleNSWand the Florey Institute of Neuroscience and Mental HealthParkvilleVICAustralia

Alexander disease is a rare neurodegenerative disorder caused by mutations in the glial fibrillary acidic protein,a type III intermediate filament protein expressed in *** early(infantile or juvenile)and adult onsets of the disease are known and,in both cases,astrocytes present characteristic aggregates,named Rosenthal *** are spread along the glial fibrillary acidic protein sequence disrupting the typical filament network in a dominant *** the presence of aggregates suggests a proteostasis problem of the mutant forms,this behavior is also observed when the expression of wild-type glial fibrillary acidic protein is ***,several isoforms of glial fibrillary acidic protein have been described to date,while the impact of the mutations on their expression and proportion has not been exhaustively ***,the posttranslational modification patterns and/or the protein-protein interaction networks of the glial fibrillary acidic protein mutants may be altered,leading to functional changes that may modify the morphology,positioning,and/or the function of several organelles,in turn,impairing astrocyte normal function and subsequently affecting *** particular,mitochondrial function,redox balance and susceptibility to oxidative stress may contribute to the derangement of glial fibrillary acidic protein mutant-expressing *** study the disease and to develop putative therapeutic strategies,several experimental models have been developed,a collection that is in constant *** fact that most cases of Alexander disease can be related to glial fibrillary acidic protein mutations,together with the availability of new and more relevant experimental models,holds promise for the design and assay of novel therapeutic strategies.

关键词： astrocytes endoplasmic reticulum stress glial fibrillary acidic protein mutants metabolism misassembly misfolding neurodegeneration oxidative stress posttranslational modifications unfolded protein response

Bone marrow-derived mononuclear stem cells in the treatment of retinal degenerations

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Neural Regeneration Research 2022年第9期17卷 1937-1944页

作者： Diego García-Ayuso Johnny Di pierdomenico David García-Bernal Manuel Vidal-Sanz María p.Villegas-p.rez Departamento de Oftalmología Facultad de MedicinaUniversidad de MurciaCampus de Ciencias de la saludMurciaSpain Instituto Murciano de Investigación Biosanitaria Hospital Virgen de la Arrixaca(IMIB-Virgen de la Arrixaca) MurciaSpain Servicio de Hematología Hospital Clínico Universitario Virgen de la ArrixacaMurciaSpain

Retinal degenerative diseases affecting the outer retina in its many forms(inherited,acquired or induced)are characterized by photoreceptor loss,and represent currently a leading cause of irreversible vision loss in the *** present,there are very few treatments capable of preventing,recovering or reversing photoreceptor degeneration or the secondary retinal remodeling,which follows photoreceptor loss and can also cause the death of other retinal ***,these diseases are nowadays one of the greatest challenges in the field of ophthalmological *** marrow derived-mononuclear stem cell transplantation has shown promising results for the treatment of photoreceptor *** cells may have the potential to slow down photoreceptor loss,and therefore should be applied in the early stages of photoreceptor ***,because of their possible paracrine effects,they may have a wide range of clinical applications,since they can potentially impact on several retinal cell types at once and photoreceptor degenerations can involve different cells and/or begin in one cell type and then affect adjacent *** intraocular injection of bone marrow derived-mononuclear stem cells also enhances the outcomes of other treatments aimed to protect ***,it is likely that future investigations may combine bone marrow derived-mononuclear stem cell therapy with other systemic or intraocular treatments to obtain greater therapeutic effects in degenerative retinal diseases.

关键词： age-related macular degeneration bone marrow stem cells intravitreal injection macroglia microglia photoreceptor degeneration retinal ganglion cells retinitis pigmentosa subretinal injection transplant

Importance of Quercus *** diversity and biomass of vascular epiphytes in a managed pine-oak forest in Southern Mexico

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Forest Ecosystems 2022年第3期9卷 366-377页

作者： Nayely Martínez-Meléndez Neptalí Ramírez-Marcial José G. García-Franco Manuel Jesús Cach-p.rez pablo Martínez-Zurimendi Departamento de Conservacion de la Biodiversidad El Colegio de la Frontera Sur(ECOSUR)San Cristobal de Las Casas29290ChiapasMexico Red de Ecología Functional Instituto de EcologíaA.C.Xalapa91073VeracruzMexico Departamento de Agricultura Sociedad y AmbienteEl Colegio de la Frontera Sur(ECOSUR)Villahermosa86280TabascoMexico

Background:Forestry management modifies the diversity,structure,and functioning of intervened *** extraction reduces tree density and basal area,leading to changes in the communities of vascular *** objective of this study was to evaluate the diversity and biomass of vascular epiphytes in Quercus trees remaining in two pine-oak forest stands that have been subjected to two stages of the Silvicultural Development Method(release cutting,and thinning)in comparison with an unharvested old-secondary forest in southern *** each treatment,richness of epiphytes present on 60 oak trees was recorded and their dry biomass *** calculated the true diversity(Hill numbers)and beta diversity using the Jaccard coefficient of similarity,and generated rank abundance curves per taxonomic epiphyte group(bromeliads,orchids,ferns and others).For each treatment,the relationships between overall diversity and epiphyte biomass to the host trees basal area were analyzed using log linear ***:We recorded a total of 67 species of epiphytes species belonging to 10 families hosted by five species of *** greatest species richness(^(0)D)was recorded in the old-secondary *** common(^(1)D)and dominant(^(2)D)species were recorded in the release cutting than in the other *** diversity and biomass were both slightly related to host tree basal *** of epiphytes was similar(60%)among treatments,although orchids,bromeliads,and other families were more diverse in the old-secondary *** bromeliad species were shared across all treatments,although orchids presented the most exclusive species in the unharvested *** bromeliad Tillandsia seleriana provided the greatest contribution to biomass in all treatments,followed by the orchid Camaridium *** linear models indicated that epiphyte diversity was significantly related to treatment,and epiphyte biomass to basal area of host ***:Alt

关键词： Bromeliads Chiapas Ferns Orchids Richness Sierra Madre Silvicultural development method(SDM)

Severe Acute Hepatitis of Unknown Origin in Children: What Do We Know Today?

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Journal of Clinical and Translational Hepatology 2022年第4期10卷 711-717页

作者： María Teresa p.rez-Gracia Antonio Tarín-pelló Beatriz Suay-García Área de Microbiología.Departamento de Farmacia Universidad Cardenal Herrera-CEU.CEU UniversitiesAlfara del Patri-arca(Valencia)Spain ESI International Chair@CEU-UCH Departamento de MatemáticasFísica y Ciencias TecnológicasUniversidad Cardenal Herrera-CEUCEU UniversitiesAlfara del Patriarca(Valencia)Spain

In May 2022, the UK International Health Regulations National Focal point notified World Health Organization of 176 cases of severe acute hepatitis of unknown etiology in children under 10 years of age. From that moment on, cases of severe acute hepatitis of unknown origin in children began to be reported in several countries. As of June 17, 2022, a total of 991 cases had been reported in 35 countries worldwide, 50 children needed a liver transplant and 28 patients died. According to information published by ECDC, 449 cases have been detected in 21 EU countries. The children were between 1 month and 16 years of age. Adenovirus was detected in 62.2% of the analyzed samples. So far, the cause of these cases is unknown and many hypotheses remain open, but hepatitis A-E viruses and COVID-19 vaccines have been ruled out. A possible hypothesis has been published to explain the cause of these cases of severe hepatitis, according to which it could be a consequence of adenovirus infection in the intestine in healthy children previously infected with SARS-CoV-2. No other clear epidemiological risk factors have been identified to date. Thus, at this time, the etiology of the current cases of hepatitis remains under active investigation.

关键词： Hepatitis Children Unknown origin SARS-CoV-2 Adenovirus

Impact of dietary supplementation of β-hydroxybutyric acid on performance, nutrient digestibility, organ development and serum stress indicators in early-weaned goat kids

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Animal Nutrition 2022年第2期9卷 16-22页

作者： Mahmoud M.Abdelsattar Einar Vargas-Bello-p.rez Yimin Zhuang Yuze Fu Naifeng Zhang Key Laboratory of Feed Biotechnology of the Ministry of Agriculture and Rural Affairs Institute of Feed Research of Chinese Academy of Agricultural Sciences Department of Animal and poultry production Faculty of Agriculture South Valley University Department of Veterinary and Animal Sciences Faculty of Health and Medical Sciences University of Copenhagen Grùnnegardsvej 3

The objective of this study was to determine the effect of dietary supplementation of β-hydroxybutyric acid(BHBA) on performance, nutrient digestibility, organ development, and serum composition in earlyweaned goat kids. Sixty-four goat kids at 30 d of age were assigned to 4 treatments in a completely randomized design: 1) control(basal diet); 2) low(basal diet with 3 g/d per animal BHBA); 3) medium(basal diet with 6 g/d per animal BHBA; and 4) high(basal diet with 9 g/d per animal BHBA). Subsequently, 48(6 kids per treatment) goat kids were randomly selected and slaughtered at 60 and 90 d of age. Compared with the control group, BHBA at low and high doses increased body weight(p p p proved organ development, especially at the lowest dose(p protein increased with age(p pared with the control group, serum ceruloplasmin increased(p p pacity and superoxide dismutase decreased with the high dose of BHBA at 90 d of age(p peroxidase and malondialdehyde increased with the high doses of BHBA(p positive for growth performance, organ development, and health status against weaning stress. Whereas high doses of BHBA in the long term could negatively affect antioxidant status.

关键词： Beta-hydroxybutyric acid Goat kid Acute-phase protein response Weaning stress Antioxidant capacity

The relation between ductility at high temperature and solid solution in Mg alloys

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Journal of Magnesium and Alloys 2022年第1期10卷 241-256页

作者： D.F.Shi C.M.Cepeda-Jiménez M.T.p.rez-prado IM DEA Materials Institute C/Eric Ka ridel228906 GetafeMadridSpain Department of Materials Science Polytechnic University of Madrid/Universidad Politecnica de MadridE.T.S.de Ingenieros de Caminos28040MadridSpain Department of physical Metallurgy CENIMCSICAv.Gregorio del Amo 828040 MadridSpain

This work investigates the effect of solid solution on ductility and on the activation of individual deformation mechanisms at moderate temperatures and at quasi-static strain rates in Mg-Zn and Mg-Al alloys. With that aim, four solid solution Mg-Zn and Mg-Al binary alloy ingots containing 1 and 2 wt.% solute atoms were subjected to hot rolling and subsequent annealing to generate polycrystals with similar average grain size and basal-type texture for each composition. The activity of the different slip systems after tensile testing at 150°C and at 250°C was evaluated in pure Mg and in the alloys by EBSD-assisted slip trace analysis. In addition, segregation of Zn and Al atoms at grain boundaries during the thermo-mechanical processing was characterized by HAADF-STEM and EDX. It was found that while the addition of Al and Zn atoms to pure Mg does not lead to major changes in the mechanical strength at the investigated temperatures, it does enhance ductility significantly, especially at 250℃. Our results show that this increase in ductility cannot be attributed to a higher activation of non-basal systems in the alloys, as reported earlier, as the incidence of non-basal systems is indeed considerably higher in pure Mg. This work suggests, on the contrary, that the ductility increase may be attributed to the presence of a more homogenous basal activity in the alloys due to a lower degree of orientation clustering, to grain boundary solute segregation, and to a higher slip diffusivity at grain interiors.

关键词： Magnesium Solute segregation Slip trace analysis Ductility Deformation mechanisms

Feasibility of the 2-point method to determine the load-velocity relationship variables during the countermovement jump exercise

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Journal of Sport and Health Science 2023年第4期12卷 544-552,F0003页

作者： Alejandro p.rez-Castilla Rodrigo Ramirez-Campillo John F.T.Fernandes Amador García-Ramos Department of physical Education and Sport Faculty of Sport SciencesUniversity of GranadaGranada 18011Spain Human performance Laboratory Department of Physical Activity SciencesUniversidad de Los LagosSantiago 5170000Chile Centro de Investigacion en Fisiologıa del Ejercicio Facultad de CienciasUniversidad MayorSantiago 8580745Chile Higher Education Sport Hartpury UniversityHartpury GL193BEUK Department of Sports Sciences and physical Conditioning Faculty of EducationUniversidad Catolica de la Santısima ConcepcionConcepcion 4090541Chile

purpose:This study aimed to examine the reliability and validity of load-velocity(L-V)relationship variables obtained through the 2-point method using different load combinations and velocity ***:Twenty men performed 2 identical sessions consisting of 2 countermovement jumps against 4 external loads(20 kg,40 kg,60 kg,and80 kg)and a heavy squat against a load linked to a mean velocity(MV)of 0.55 m/s(load_(0.55)).The L-V relationship variables(load-axis intercept(L_(0)),velocity-axis intercept(v_(0)),and area under the L-V relationship line(A_(line)))were obtained using 3 velocity variables(MV,mean propulsive velocity(MpV),and peak velocity)by the multiple-point method including(20-40-60-80-load_(0.55))and excluding(20-40-60-80)the heavy squat,as well as from their respective 2-point methods(20-load_(0.55)and 20-80).Results:The L-V relationship variables were obtained with an acceptable reliability(coefncient of variation(CV)≤7.30%;intra-class correlation coefficient>0.63).The reliability of L_(0)and v_(0)was comparable for both methods(CV_(ratio)(calculated as higher value/lower value):1.11-1.12),but the multiple-point method provided Al_(ine)with a greater reliability(CV_(ratio)=1.26).The use of a heavy squat provided the L-V relationship variables with a comparable or higher reliability than the use of a heavy countermovement jump load(CV_(ratio):1.06-1.19).The peak velocity provided the load-velocity relationship variables with the greatest reliability(CV_(ratio):1.15-1.86)followed by the MV(CV_(ratio):1.07-1.18),and finally the *** 2-point methods only revealed an acceptable validity for the MV and MpV(effect size≤0.19;pearson s product-moment correlation coefficient≥0.96;Lin's concordance correlation coefficient≥0.94).Conclusion:The 2-point method obtained from a heavy squat load and MV or MpV is a quick,safe,and reliable procedure to evaluate the lower-body maximal neuromuscular capacities through the L-V relationship.

关键词： Force-velocity relationship Mean velocity Multiple-point method peak velocity Velocity-based training

The short form of the SUR1 and its functional implications in the damaged brain

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Neural Regeneration Research 2022年第3期17卷 488-496页

作者： Iván Alquisiras-Burgos Javier Franco-p.rez Moisés Rubio-Osornio penélope Aguilera Laboratorio de patología Vascular Cerebral Instituto Nacional de Neurología y Neurocirugía“Manuel Velasco Suárez”CDMXMexico Laboratorio de Formación Reticular Instituto Nacional de Neurología y Neurocirugía“Manuel Velasco Suárez”CDMXMexico Laboratorio Experimental de Enfermedades Neurodegenerativas Instituto Nacional de Neurología y Neurocirugía“Manuel Velasco Suárez”CDMXMexico.

Sulfonylurea receptor(SUR) belongs to the adenosine 5′-triphosphate(ATp)-binding cassette(ABC) transporter family;however,SUR is associated with ion channels and acts as a regulatory subunit determining the opening or closing of the ***8 and Abcc9 genes code for the proteins SUR1 and SUR2,*** SUR1 transcript encodes a protein of 1582 amino acids with a mass around 140–177 k Da expressed in the pancreas,brain,heart,and other *** is well known that SUR1 assembles with Kir6.2 and TRpM4 to establish K_(ATp) channels and non-selective cation channels,***8 and 9 are alternatively spliced,and the resulting transcripts encode different isoforms of SUR1 and SUR2,which have been detected by different experimental ***,the use of binding assays to sulfonylureas and Western blotting has allowed the detection of shorter forms of SUR(-65 k Da).Identity of the SUR1 variants has not been clarified,and some authors have suggested that the shorter forms are ***,immunoprecipitation assays have shown that SUR2 short forms are part of a functional channel even coexisting with the typical forms of the receptor in the *** evidence confirms that the structure of the short forms of the SURs is fully functional and does not lose the ability to interact with the *** structural changes in short forms of SUR modify its affinity to ATp,regulation of its expression might represent an advantage in pathologies where ATp concentrations decrease and a therapeutic target to induce ***,the expression of SUR1 variants might be induced by conditions associated to the decrease of energetic substrates in the brain(*** stroke and epilepsy).In this review,we want to contribute to the knowledge of SUR1 complexity by analyzing evidence that shows the existence of short SUR1 variants and its possible implications in brain function.

关键词： brain edema epilepsy parkinson's disease stroke sulfonylurea receptor 1 SUR1 traumatic brain injury TRpM4

Endoscopic ultrasound-guided gastroenterostomy for gastric outlet obstruction in Mexico

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World Journal of Surgical procedures 2024年第3期14卷 15-20页

作者： Massiel Madelin Rosario-Morel Rodrigo Soto-Solis Katia picazo-Ferrera Miriam Idalia Torres-Ruiz JoséAlberto Estradas-Trujillo Mario Alberto Gallardo-Ramírez Gerardo Akram Darwich-del Moral Luis Ariel Waller-González Department of Endoscopy Centro Médico Nacional“20 de Noviembre”ISSSTEMexico City 03229Mexico Department of Gastroenterology HospitalÁngeles PedregalMexico City 10700Mexico Department of Endoscopy Centro Médico ABCMexico City 01120Mexico Department of Endoscopy Hospital General de México“Dr.Eduardo Liceaga”Mexico City 06720Mexico

BACKGROUND Endoscopic ultrasound-guided gastroenterostomy(EUS-GE)has recently emerged as an alternative treatment for gastric outlet obstruction(GOO)in selected *** To report the initial experience of EUS-GE in patients with *** This study was a retrospective,observational,multicenter study in which the data from 10 patients who underwent EUS-GE due to GOO between September 2021 and May 2023 were *** analyzed technical success,clinical success,adverse events,and *** success was defined as adequate positioning and deployment of the *** success was defined as the patient’s ability to tolerate oral intake without vomiting 7 d after the *** adverse events were *** Eleven procedures in 10 patients with GOO were *** mean age of the patients was 67.5 years(range:56-77 years).Malignant GOO was present in 9 *** success was achieved in 9/11 procedures(82%).Among them,clinical success was achieved in 9 patients(100%).Adverse events occurred in 1 patient(9%).The median survival was 3 months(n=7;range:1-8 months).CONCLUSION EUS-GE is a feasible therapeutic option in the treatment of GOO.

关键词： Endoscopic ultrasound-guided gastroenterostomy Gastric outlet obstruction Lumen apposing metal stent Interventional endoscopic ultrasound Gastrojejunostomy Duodenal stenting