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Prion-like domain disease-causing mutations and misregulation of alternative splicing relevance in limb-girdle muscular dystrophy (LGMD) 1G

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Neural Regeneration Research 2020年第12期15卷 2239-2240页

作者： cristina Batlle salvador Ventura Departament de Bioquímica i Biologia Molecular and Institut de Biotecnologia i Biomedicina Universitat Autònoma de BarcelonaBellaterraSpain

Human prion-like proteins often correspond to nucleic acid binding proteins,displaying both globular domains and long intrinsically disordered regions(IDRs)(Harrison and Shorter,2017).Their IDRs are of low complexity and resemble in amino acid composition to the disordered yeast prion domains,being usually enriched in Gln and Asn residues and depleted in hydrophobic and charged ***,these sequence stretches are named prion-like domains(PrLDs).Prion-like proteins can aggregate into amyloid fibrils,which can accommodate incoming protein monomers,propagating thus the polymeric fold,both processes being。

关键词： dystrophy muscular amyloid

The 3x + 1 conjecture, a Direct Path

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American Journal of computational Mathematics 2023年第2期13卷 350-355页

作者： salvador Bermúdez Gómez Instituto Politécnico Nacional SEPI-ESIME-Zacatenco Mexico City México

The 3x + 1 problem, is a math problem that has baffled mathematicians for over 50 years. It’s easy to explain: take any positive number, if it’s even, divide it by 2;if it’s odd, multiply it by 3 and add 1. Repeat this process with the resulting number, and the conjecture says that you will eventually reach 1. Despite testing all starting values up to an enormous number, no one has proved the conjecture is true for all possible starting values. The problem’s importance lies in its simplicity and difficulty, inspiring new ideas in mathematics and advancing fields like number theory, dynamical systems, and computer science. Proving or disproving the conjecture would revolutionize our understanding of math. The presence of infinite sequences is a matter of question. To investigate and solve this conjecture, we are utilizing a novel approach involving the fields of number theory and computer science.

关键词： 3x + 1 collatz Solved computer Science Number Theory New Algo-rithm

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Neural Regeneration Research 2021年第1期16卷 123-124页

作者： carlos Bueno salvador Martínez Instituto de Neurociencias de Alicante(UMH-cSIc) San JuanAlicanteSpain

Human neurological disorders and spinal cord injuries are caused by a loss of neurons and glial cells in the brain or spinal *** proof-ofprinciple of cell therapy is that replacing damaged cells with new healthy ones will restore the lost *** and manipulating autologous neural stem cells(NScs)could provide an ideal source of cells for use in cell replacement and the transfer of genes to a diseased central nervous *** are immature cells present,not only during embryonic development,but also in the adult brain of all mammalian species,including *** presence of NScs in the adult mammalian brain has been described in two neurogenic niches,the ventricular-subventricular zone(V-SVZ)of the anterolateral ventricle wall and the subgranular zone of the hippocampal dentate ***,NScs are defined as self-renewing multipotent cells that can generate neurons,astrocytes and *** major barrier to isolating adult NScs in humans is the inaccessibility of living *** this reason,an enormous effort has been made to derive neurons from human adult stem cells isolated from various tissue sources.

关键词： injuries ventricular adult

State and perspectives on flavonoid neuroprotection against aminochrome-induced neurotoxicity

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Neural Regeneration Research 2021年第9期16卷 1797-1798页

作者： Victor Silva Juan Segura-aguilar Laboratory of Neurochemistry and cell Biology Department of Biochemistry and BiophysicsInstitute of Health SciencesFederal University of Bahia40110-100SalvadorBahiaBrazil Molecular and clinical Pharmacology ICBMFaculty of MedicineUniversity of ChileChile

Parkinson's disease(PD) motor symptoms are induced by the loss of dopaminergic neurons containing neuromelanin in the nigrostriatal system. The exact mechanism that triggers the degeneration of the nigrostriatal neurons is still unknown but there is general consensus in the scientific community that mitochondrial dysfunction, alpha-synuclein aggregation to neurotoxic oligomers, protein degradation dysfunction of both lysosomal and proteasomal systems, endoplasmic reticulum stress.

关键词： degeneration reticulum aggregation

Using electricity options to hedge against financial risks of power producers

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Journal of Modern Power Systems and clean Energy 2013年第2期1卷 101-109页

作者： salvador PINEDA Antonio J.cONEJO center for Electric Technology Department of Electrical EngineeringTechnical University of DenmarkKgs.LyngbyDenmark University of castilla-La Mancha Ciudad RealSpain

As a consequence of competition in electricity markets,a wide variety of financial derivatives have emerged to allow market agents to hedge against *** options and forward contracts constitute adequate instruments to manage the financial risks pertaining to price volatility or unexpected unit failures faced by power producers.A multi-stage stochastic model is described in this tutorial paper to determine the optimal forward and option contracting decisions for a risk-averse power *** key features of electricity options to reduce both price and availability risks are illustrated by using two examples.

关键词： Price risk Availability risk Stochastic programming Forward contracts Electricity options

Inducing α-synuclein compaction: a new strategy for inhibiting α-synuclein aggregation?

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Neural Regeneration Research 2019年第11期14卷 1897-1898页

作者： Francisca Pinheiro salvador Ventura Institut de Biotecnologia i Biomedicina Universitat Autonoma de BarcelonaBellaterraSpainDepartament de Bioquimica i Biologia MolecularUniversitat Autonoma de BarcelonaBellaterraSpain

Proteins might misfold during translation and folding or even once they are in their native states, due to stochastic fluctuations, destabilizing mutations or cellular stress. Aberrant protein species are usually detected and either refolded or cleared by the protein quality control machinery (ciechanover and Kwon, 2015). When misfolded protein conformers cannot be degraded, they tend to self-assemble to form aggregates, a characteristic of many neurodegenerative diseases.

关键词： inhibitingα-synuclein aggregation native states protein quality control machinery

Mine tailings storage dams modify upstream headwater fish assemblages

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Water Biology and Security 2023年第2期2卷 32-42页

作者： Gilberto N.salvador Robert M.Hughes Fabio Vieira Raphael Ligeiro Luciano F.A.Montag Ecology and conservation Laboratory(LABEcO) Federal University of ParaBiological Sciences InstituteRua Augusto Correa01GuamaCaixaBelemPA479Brazil Fish Ecology and conservation Laboratory(Eco-Peixes) Federal University of Minas GeraisBiological Sciences InstituteAv.Antonio Carlos6627Belo HorizonteMGCEP:31.270-901Brazil Amnis Opes Institute 2895 SE GlennCorvallisOR97333USA Department of Fisheries Wildlife&Conservation SciencesOregon State UniversityCorvallisOR97331USA Zoology Department Federal University of Minas GeraisBiological Sciences InstituteAv.Antonio Carlos6627CEP:31.270-901Belo HorizonteMGBrazil

Riverscape connectivity is a critically important component determining the ecological condition of lotic *** evaluated changes in fish assemblages caused by the loss of connectivity by mine tailings storage dams(TSDs),hypothesizing that headwater fish assemblages are restructured by TSDs located downstream,even though the upstream habitats are not *** used standard methods to collect fish in 24 first to third order sites,with half draining to TSDs(dammed)and the other half free from this impact(undammed).To identify differences between treatments,we used PERMANOVA to test both environmental variables and ichthyofauna composition(Bray-curtis similarity index)and evaluated the biological metrics that most influenced assemblage composition *** expected,we observed no difference between treatments for environmental variables,but we did observe differences in fish assemblage *** also observed five metrics with lower values in dammed streams(richness and abundance of intolerant species,siluriform richness,and abundance of Pareiorhaphis *** and Trichomycterus brasiliensis)and two metrics with higher values in dammed streams(perciform richness and abundance of Oligosarcus argenteus).We believe these changes resulted from of stream fragmentation by TSDs,in addition to source-sink mechanisms and conclude that mine TSDs located downstream change headwater fish assemblages,an impact often neglected in biomonitoring and bioassessment studies.

关键词： Habitat fragmentation Source-sink dynamics Iron ore mining Rio Doce basin

Nitric oxide and hydrogen sulfide share regulatory functions in higher plant events

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BIOcELL 2022年第1期46卷 1-5页

作者： FRANcIScO J.cORPAS salvador GONZÁLEZ-GORDO MARTA RODRÍGUEZ-RUIZ MARÍA A.MUÑOZ-VARGAS JOSÉM.PALMA Group of Antioxidants Free Radicals and Nitric Oxide in BiotechnologyFood and AgricultureDepartment of BiochemistryCell and Molecular Biology of PlantsEstación Experimental del Zaidín(Spanish National Research CouncilCSIC)Granada18008Spain

Nitric oxide(NO)and hydrogen sulfide(HS)are two molecules that share signaling properties in plant and animal cells NO and H2S originate two farmilies of de rived mol ecules designated reactive nitrogen and sulfur species(RNS and RSS,respectively).These molecules are responsible for certain protein regulatory processes through posttranslational modifications(PTMs),being the most remarkable S nitrosation and persufidation,which afect the thiol group of cysteine *** and H2S can also exert regulatory functions due to their interaction through the iron present in proteins that contain heme groups or iron-sulfur dlusters,as reported mainly in animal ***,the available information in plant cells is still very limited thus *** higher plants,NO and H2S are involved in a myriad of physiological events from seed germination to fruit ripening,but also the mec hanism of response to biotic and abiotic stress *** vie wpoint manuscript highlights the functional regulatory parllelism of these two molecules which also interact with the metabolism of reactive oxygen species(ROS)in plant cells.

关键词： Ascorbate peroxidase Hydrogensulfide Nitricoxide Persulfidation S-nitrosation Signaling

Theobroma cacao improves bone growth by modulating defective ciliogenesis in a mouse model of achondroplasia

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Bone Research 2022年第1期10卷 137-149页

作者： Ludovic Martin Nabil Kaci catherine Benoist-Lasselin Marine Mondoloni Suzanne Decaudaveine Valentin Estibals Maxence cornille Léa Loisay Justine Flipo Benoît Demuynck Maria de la Luz cádiz-Gurrea Florent Barbault salvador Fernández-Arroyo Laurent Schibler Antonio Segura-carretero Emilie Dambroise Laurence Legeai-Mallet Universitéde Paris Imagine InstituteLaboratory of Molecular and Physiopathological Bases of OsteochondrodysplasiaINSERM UMR 1163F‑75015ParisFrance Inovarion ParisFrance Department of Analytical chemistry University of GranadaGranadaSpain Research and Development of Functional Food centre(cIDAF) GranadaSpain Universitéde Paris ITODYSCNRSUMR 708615 rue J-A de BaïfParisFrance Biomedical Research Unit Medicine and Surgery DepartmentRovira i Virgili UniversityTarragonaSpain ALLIcE Maison Nationale des EleveursParisFrance

A gain-of-function mutation in the fibroblast growth factor receptor 3 gene(FGFR3)results in achondroplasia(AcH),the most frequent form of *** activation of FGFR3 impairs bone formation and elongation and many signal transduction *** of new and relevant compounds targeting the FGFR3 signaling pathway is of broad importance for the treatment of AcH,and natural plant compounds are prime drug candidate ***,we found that the phenolic compound(-)-epicatechin,isolated from Theobroma cacao,effectively inhibited FGFR3’s downstream signaling *** analysis in an Fgfr3 mouse model showed that ciliary mRNA expression was modified and influenced significantly by the Indian hedgehog and PKA pathways.(-)-Epicatechin is able to rescue mRNA expression impairments that control both the structural organization of the primary cilium and ciliogenesis-related *** femurs isolated from a mouse model(Fgfr3^(Y367c/+))of AcH,we showed that(-)-epicatechin eliminated bone growth impairment during 6 days of ex vivo *** vivo,we confirmed that daily subcutaneous injections of(-)-epicatechin to Fgfr3^(Y367c/+) mice increased bone elongation and rescued the primary cilium defects observed in *** modification to the primary cilia promoted the typical columnar arrangement of flat proliferative chondrocytes and thus enhanced bone *** results of the present proof-of-principle study support(-)-epicatechin as a potential drug for the treatment of AcH.

关键词： inhibited arrangement eliminated