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Clinical and radiographic features of Hutchinson-Gilford progeria syndrome: A case report

World Journal of Clinical Cases 2014年第3期2卷 67-71页

作者： daniel berretta alves Juliana Melo Silva Tatiany Oliveira Menezes Rosely Santos Cavaleiro Fabrício Mesquita Tuji Marcio Ajudarte Lopes Alexandre Augusto Zaia Ricardo Della Coletta Department of Radiology Esperanca Institute of Higher Learning Santarém-Pará 68040-100 Brazil Department of Endodontics Federal University of Pará Belém-Pará 66055-240 Brazil Department of Dentistry for Patients with Special Needs Federal University of Pará Belém-Pará 66055-240 Brazil Department of Oral and Maxillofacial Pathology Federal University of Pará Belém-Pará 66055-240 Brazil Department of Oral Diagnosis School of Dentistry State University of Campinas Piracicaba-Sao Paulo 13083-970 Brazil Department of Restorative Dentistry School of Dentistry State University of Campinas Piracicaba-Sao Paulo 13083-970 Brazil

Hutchinson-Gilford progeria syndrome(HGPS) is a rare dysmorphic syndrome characterized by several features of premature aging with clinical involvement of the skin, bones, and cardiovascular system. HGPS has an estimated incidence of one in four million to one in eight million births. The main clinical features of HGPS include short stature, craniofacial dimorphism, alopecia, bone fragility, and cardiovascular disorders. The most frequent cause of death is myocardial infarction at a mean age of 13 years old. Dental manifestations include delayed development and eruption of teeth, discoloration, crowding and rotation of teeth, and displaced teeth. Cone beam computed tomography images revealed the absence of the sphenoid, frontal, and maxillary sinus, flattening of the condyles and glenoid fossa, and bilateral hypoplasia of the mandibular condyles. The disease is caused by mutations in lamin A/C(LMNA). Here, we present a case report of an 11-year-old boy with classical features of HGPS, which was caused by a de novo germ-line mutation(C1824T, G608G) in exon 11 of the LMNA gene. Some uncommon HGPS-associated features in our patient, such as alterations in the facial sinuses and hypoplasia of the condyles, contributed to the expansion of the phenotypic spectrum of this syndrome from a dentomaxillofacial perspective.

关键词： Cone beam computed tomography LMNA mutation Craniofacial anomalies Temporomandibular joint

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Amyloid-β-induced disruption of axon-initial-segment mitochondria localization:consequences for TAU missorting in Alzheimer's disease pathology

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Neural Regeneration Research 2025年第5期20卷 1407-1408页

作者： daniel Adam Felix Langerscheidt Hans Zempel Institute of Human Genetics University Hospital CologneCologneGermany Center for Molecular Medicine Cologne(CMMC) University of CologneCologneGermany

TAU is a neuronal microtubule-associated protein preferentially located in *** a battery of neurodegenerative diseases termed"tauopathies,"including Alzheimer's disease (AD),TAU is missorted and abnormally phosphorylated,leading to filamentous accumulations of hyperphosphorylated TAU,a pathological hallmark and potential disease driver of AD and related tauopathies (Zempel,2024).

关键词： Alzheimer tau diseases

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Wafer‑Scale Ag_(2)S‑Based Memristive Crossbar Arrays with Ultra‑Low Switching‑Energies Reaching Biological Synapses

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Nano-Micro Letters 2025年第3期17卷 328-339页

作者： Yuan Zhu Tomas Nyberg Leif Nyholm daniel Primetzhofer Xun Shi Zhen Zhang Division of Solid‑State Electronics Department of Electrical EngineeringUppsala University75121 UppsalaSweden Department of Chemistry Uppsala UniversityUppsalaSweden Department of Physics and Astronomy Uppsala UniversityUppsalaSweden State Key Laboratory of High Performance Ceramics and Superfine Microstructure Shanghai Institute of CeramicsChinese Academy of SciencesShanghai 200050People’s Republic of China

Memristive crossbar arrays(MCAs)offer parallel data storage and processing for energy-efficient neuromorphic ***,most wafer-scale MCAs that are compatible with complementary metal-oxide-semiconductor(CMOS)technology still suffer from substantially larger energy consumption than biological synapses,due to the slow kinetics of forming conductive paths inside the memristive *** we report wafer-scale Ag_(2)S-based MCAs realized using CMOS-compatible processes at temperatures below 160℃.Ag_(2)S electrolytes supply highly mobile Ag+ions,and provide the Ag/Ag_(2)S interface with low silver nucleation barrier to form silver filaments at low energy *** further enhancing Ag+migration in Ag_(2)S electrolytes via microstructure modulation,the integrated memristors exhibit a record low threshold of approximately−0.1 V,and demonstrate ultra-low switching-energies reaching femtojoule values as observed in biological *** low-temperature process also enables MCA integration on polyimide substrates for applications in flexible ***,the intrinsic nonidealities of the memristive units for deep learning can be compensated by employing an advanced training *** impressive accuracy of 92.6%in image recognition simulations is demonstrated with the MCAs after the *** demonstrated MCAs provide a promising device option for neuromorphic computing with ultra-high energy-efficiency.

关键词： Wafer-scale Ag_(2)S films Reactive sputter Silver nucleation Ag^(+)migration Energy-efficient neuromorphic computing

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Vascular endothelial growth factor:a double-edged sword in the development of white matter lesions

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Neural Regeneration Research 2025年第1期20卷 191-192页

作者： Narek Manukjan daniel Fulton Zubair Ahmed WMatthijs Blankesteijn Sébastien Foulquier Department of Pharmacology and Toxicology Maastricht UniversityMaastrichtThe Netherlands CARIM-School for Cardiovascular Diseases Maastricht UniversityMaastrichtThe Netherlands Neuroscience and Ophthalmology Institute of Inflammation and AgeingUniversity of BirminghamEdgbastonBirminghamUK Centre for Trauma Sciences Research University of BirminghamEdgbastonBirminghamUK MHeNs-School for Mental Health and Neuroscience Maastricht UniversityMaastrichtThe Netherlands Department of Neurology Maastricht University Medical CenterMaastrichtThe Netherlands

As the population ages,the burden of age-related diseases becomes ***,over 55 million people suffer from dementia worldwide,with Alzheimer’s disease being the most common ***,it is becoming clearer that underlying vascular pathology such as cerebral small vessel disease(cSVD)may be a more detrimental cause for dementia(Cuadrado-Godia et al.,2018).It is estimated that 10%-30%of the elderly population and 35%-90%of all dementia patients exhibit signs of *** term cSVD refers to pathology affecting the small vessels of the brain,which can lead to lacunar cerebral infarcts,enlarged perivascular spaces,and cortical hemorrhages(Cuadrado-Godia et al.,2018).CSVD is often associated with cognitive decline,gait problems,and dementia(Cuadrado-Godia et al.,2018).

关键词： pathology becoming white

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Complement-dependent neuroinflammation in spinal cord injury:from pathology to therapeutic implications

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Neural Regeneration Research 2025年第5期20卷 1324-1335页

作者： Hassan Saad Bachar El Baba Ali Tfaily Firas Kobeissy Juanmarco Gutierrez Gonzalez daniel Refai Gerald R.Rodts Christian Mustroph David Gimbel Jonathan Grossberg daniel L.Barrow Matthew F.Gary Ali M.Alawieh Department of Neurosurgery Emory University School of MedicineAtlantaGAUSA Department of Neurology Yale UniversityNew HavenCTUSA Center for Neurotrauma Multiomics&Biomarkers(CNMB)Morehouse School of MedicineAtlantaGAUSA

Spinal cord injury remains a major cause of disability in young adults,and beyond acute decompression and rehabilitation,there are no pharmacological treatments to limit the progression of injury and optimize recovery in this *** the thorough investigation of the complement system in triggering and propagating cerebral neuroinflammation,a similar role for complement in spinal neuroinflammation is a focus of ongoing *** this work,we survey the current literature investigating the role of complement in spinal cord injury including the sources of complement proteins,triggers of complement activation,and role of effector functions in the *** study relevant data demonstrating the different triggers of complement activation after spinal cord injury including direct binding to cellular debris,and or activation via antibody binding to damage-associated molecular *** effector functions of complement have been implicated in spinal cord injury,and we critically evaluate recent studies on the dual role of complement anaphylatoxins in spinal cord injury while emphasizing the lack of pathophysiological understanding of the role of opsonins in spinal cord *** this pathophysiological review,we systematically review the different translational approaches used in preclinical models of spinal cord injury and discuss the challenges for future translation into human *** review emphasizes the need for future studies to dissect the roles of different complement pathways in the pathology of spinal cord injury,to evaluate the phases of involvement of opsonins and anaphylatoxins,and to study the role of complement in white matter degeneration and regeneration using translational strategies to supplement genetic models.

关键词： complement neuroinflammation neuroplasticity regeneration spinal cord injury targeted therapy

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Regulator of G protein signaling 6 mediates exercise-induced recovery of hippocampal neurogenesis,learning,and memory in a mouse model of Alzheimer’s disease

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Neural Regeneration Research 2025年第10期20卷 2969-2981页

作者： Mackenzie M.Spicer Jianqi Yang daniel Fu Alison N.DeVore Marisol Lauffer Nilufer S.Atasoy Deniz Atasoy Rory A.Fisher Department of Neuroscience and Pharmacology University of Iowa Carver College of MedicineIowa CityIAUSA Iowa Neuroscience Institute University of Iowa Carver College of MedicineIowa CityIAUSA Interdisciplinary Graduate Program in Molecular Medicine University of Iowa Carver College of MedicineIowa CityIAUSA Neural Circuits and Behavior Core University of Iowa Carver College of MedicineIowa CityIAUSA

Hippocampal neuronal loss causes cognitive dysfunction in Alzheimer’s *** hippocampal neurogenesis is reduced in patients with Alzheimer’s *** stimulates adult hippocampal neurogenesis in rodents and improves memory and slows cognitive decline in patients with Alzheimer’s ***,the molecular pathways for exercise-induced adult hippocampal neurogenesis and improved cognition in Alzheimer’s disease are poorly ***,regulator of G protein signaling 6(RGS6)was identified as the mediator of voluntary running-induced adult hippocampal neurogenesis in ***,we generated novel RGS6fl/fl;APP_(SWE) mice and used retroviral approaches to examine the impact of RGS6 deletion from dentate gyrus neuronal progenitor cells on voluntary running-induced adult hippocampal neurogenesis and cognition in an amyloid-based Alzheimer’s disease mouse *** found that voluntary running in APP_(SWE) mice restored their hippocampal cognitive impairments to that of control *** cognitive rescue was abolished by RGS6 deletion in dentate gyrus neuronal progenitor cells,which also abolished running-mediated increases in adult hippocampal *** hippocampal neurogenesis was reduced in sedentary APP_(SWE) mice versus control mice,with basal adult hippocampal neurogenesis reduced by RGS6 deletion in dentate gyrus neural precursor ***6 was expressed in neurons within the dentate gyrus of patients with Alzheimer’s disease with significant loss of these RGS6-expressing ***,RGS6 mediated voluntary running-induced rescue of impaired cognition and adult hippocampal neurogenesis in APP_(SWE) mice,identifying RGS6 in dentate gyrus neural precursor cells as a possible therapeutic target in Alzheimer’s disease.

关键词： adult hippocampal neurogenesis Alzheimer’s disease dentate gyrus exercise learning/memory neural precursor cells regulator of G protein signaling 6(RGS6)

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Studying Formability Limits By Combining Conventional and Incremental Sheet Forming Process

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Chinese Journal of Mechanical Engineering 2021年第4期34卷 78-89页

作者： Fabio Andre Lora daniel Fritzen Ricardo alves de Sousa Lirio Schaffer Department of Materials Engineering Federal University of Recôncavo da BahiaCampus CETENSFeira de SantanaBahia 44085-132Brazil SATC Uni-versity CriciumaBrazil Center of Mechanical Technology and Automation Department of Mechanical EngineeringUniversity of Aveiro3810-193 AveiroPortugal Federal University of Rio Grande do Sul Porto AlegreBrazil

In this work it is assessed the potential of combining conventional and incremental sheet forming processes in a same sheet of *** so-called hybrid forming approach is performed through the manufacture of a pre-forming by conventional forming,followed by incremental sheet *** main objective is analyzing strain *** pre-forming induced in the conventional forming stage will determine the strain paths,directly influencing the strains produced by the incremental *** conduct the study,in the conventional processes,strains were imposed in three different ways with distinct true *** the incremental stage,the pyramid strategy was adopted with different wall *** the experiments,the true strains and the final geometries were *** simulation was also employed for the sake of comparison and correlation with the measured *** could be observed that single-stretch pre-strain was directly proportional to the maximum incremental strains achieved,whereas samples subjected to biaxial pre-strain influenced the formability according to the degree of pre-strain ***-strain driven by the prior deep-drawing operation did not result,in this particular geometry,in increased formability.

关键词： Hybrid forming process Incremental sheet forming Strain paths Numerical simulation

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On the wavelet analysis of cutting forces for chatter identification in milling

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Advances in Manufacturing 2017年第2期5卷 130-142页

作者： Cesar Giovanni Cabrera Anna Carla Araujo daniel alves Castello Department of Mechanical Engineering UniversidadeFederal do Rio de Janeiro Rio de Janeiro Brazil

Chatter vibrations in machining operations affect surface finishing and tool behaviour, particularly in the end-milling of aluminum parts for the aerospace industry. This paper presents a methodological approach to identify chatter vibrations during manufacturing processes. It relies on wavelet analyses of cutting force signals during milling operations. The cutting-force signal is first decomposed into an approximation/trend sub-signal and detailed subsignals, and it is then re-composed using modified subsignals to reduce measurement noise and strengthen the reference peak forces. The reconstruction of the cuttingforce signal is performed using a wavelet denoising pro- cedure based on a hard-thresholding method. Four experimental configurations were set with specific cutting parameters using a workpiece specifically designed to allow experiments with varying depths of cut. The experimental results indicate that resultant force peaks （after applying the threshold to the detailed sub-signals） are related to the presence of chatter, based on the increased correlation of such peaks and the surface roughness profiles, thereby reinforcing the applicability of the proposed method. The results can be used to control the online occurrence of chatter in end-milling processes, as the method does not depend on the knowledge of cutting geometry nor dynamic parameters.

关键词： Cutting force End milling Chatter Waveletfilter

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The crossroads of receptor-mediated signaling and endocytosis in plants

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Journal of Integrative Plant Biology 2018年第9期60卷 827-840页

作者： Lucas alves Neubus Claus daniel V.Savatin Eugenia Russinova Department of Plant Biotechnology and Bioinformatics Ghent University9052 GhentBelgium Center for Plant Systems Biology VIB9052 GhentBelgium

Plants deploy numerous plasma membrane receptors to sense and rapidly react to environmental changes. Correct localization and adequate protein levels of the cell-surface receptors are critical for signaling activation and modulation of plant development and defense against pathogens. After ligand binding, receptors are internalized for degradation and signaling attenuation. However, one emerging notion is that the Iigand-induced endocytosis of reCeptor complexes is important for the signal duration, amplitude, and specificity. Recently, mutants of maior endocytosis players, including clathrin and dynamin, have been shown to display defects in activation of a subset of signal transduction pathways, implying that signaling in plants might not be solely restricted to the plasma membrane. Here, we summarize the up-to-date knowledge of receptor complex endocytosis and its effect on the signaling outcome, in the context of plant development and immunity.

关键词： The crossroads of receptor-mediated signaling and endocytosis in plants BRI PM

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Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

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World Journal of Gastroenterology 2016年第28期22卷 6559-6564页

作者： Cláudio Martins Cristina Teixeira Suzane Ribeiro daniel Trabulo Cláudia Cardoso Joao Mangualde Ricardo Freire élia Gamito Ana Luísa alves Isabelle Cremers Cecília alves Anabela Neves Ana Paula Oliveira Department of Gastroenterology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal Department of Pathology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal Department of Hematology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal

Mastocytosis is a clonal neoplastic disorder of the mast cells(MC) that can be limited to the skin(cutaneous mastocytosis) or involve one or more extracutaneous organs(systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis(ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

关键词： Systemic mastocytosis Mast cells Non-cirrhotic portal hypertension Upper gastrointestinal bleeding Cladribine

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