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Cryptic exon inclusion in TDP-43 proteinopathies:opportunities and challenges

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Neural Regeneration Research 2025年第7期20卷 2003-2004页

作者： Lorena decker Sonja Menge Axel Freischmidt Department of Neurology Ulm UniversityUlmGermany

TDP-43 proteinopathies and cryptic exons:Transactive response DNA binding protein of 43 kDa(TDP-43)is a ubiquitously expressed RNA/DNA binding protein crucial for coding and non-coding RNA metabolism including transcription,splicing,transport,translation,and ***-43 shuttles between the nucleus and cytoplasm,but is predominantly localized in the *** diseases(NDs)may be accompanied by nuclear loss and possible cytoplasmic accumulation and aggregation of TDP-43 in vulnerable neurons and *** neuropathology is the hallmark of most individuals suffering from amyotrophic lateral sclerosis(ALS),frontotemporal dementia(FTD)with TDP-43-immunoreactive pathology(FTD-TDP),limbic-predominant age-related TDP-43 encephalopathy(LATE)and Perry syndrome,but also coexists with the primary pathology in subsets of patients suffering from other NDs,such as Alzheimer’s disease,Lewy body dementias,or Huntington’s *** in the gene encoding TDP-43(TARDBP)are the cause of ALS and/or FTD in some rare cases substantiating the importance of this protein in aging *** is still controversial if loss of nuclear,or increased cytoplasmic and/or aggregating TDP-43 is more harmful to neurons(Nag and Schneider,2023).Recently,the role of nuclear TDP-43 in repressing the inclusion of intronic sequences,named cryptic exons(CEs),into mature mRNAs gained much attention.

关键词： Huntington metabolism TDP

Restoring expression of Stathmin-2:a novel strategy to treat TDP-43 proteinopathies

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Signal Transduction and Targeted Therapy 2023年第8期8卷 3553-3554页

作者： Sonja Menge Lorena decker Axel Freischmidt Department of Neurology Ulm University89081 UlmGermany

In a recent study published in Science,Baughn et *** that TDP-43 acts as a steric block in STMN2 pre-mRNA processing preventing inclusion of a deleterious cryptic exon,and present strategies for substituting this function to compensate for the loss of nuclear TDP-43 in a variety of neurodegenerative diseases(NDs)including amyotrophic lateral sclerosis(ALS).1 Stathmin-2(STMN2)is a microtubule-associated protein speci-fically expressed in neurons and required for axon outgrowth and maintenance,as well as for axon regeneration after injury in vitro.

关键词： Stathmin diseases

Measurement of bullet impact conditions using automated in-flight photography system

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维普期刊数据库

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Defence Technology（防务技术） 2017年第4期13卷 288-294页

作者： Ryan decker Marco Duca Shawn Spickert-Fulton Armament Research Design and Engineering Center 94 Ramsey Avenue Picatinny Arsenal NJ 07806 USA

Knowledge of impact conditions is critical to evaluating the terminal impact performance of a *** a small caliber bullet,in-flight velocity has been precisely measured for decades using detection screens,but accurately quantifying the orientation of the bullet on a target has been more *** report introduces the Automated Small-Arms Photogrammetry(ASAP)analysis method used to measure,model,and predict the orientation of a small caliber bullet before reaching an impact *** uses advanced hardware developed by Sydor Technologies to record a series of infrared digital *** images(four orthogonal pairs)are processed using computer vision algorithms to quantify the orientation of the projectile and re-project its precise position and orientation into a three-dimensional muzzle-fixed coordinate *** epicyclic motion model is fit to the measured data,and the epicyclic motion is extrapolated to the target *** results are fairly immediate and may be reviewed during ***-out demonstrations have shown that the impact-angle prediction capability is less than six hundredths of a degree for the 5.56 mm ball round ***:Yaw,Terminal ballistics,Exterior ballistics,Test&evaluation,Computer vision,Image processing,Angle of

关键词： Terminal ballistics Exterior ballistics Test & evaluation Computer vision Image processing Angle of attack

Volcanoes Robert decker and Barbara decker 3rd ed.

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Central nervous system manifestations due to iatrogenic adrenal insuffi ciency in a Ewing sarcoma patient

1998年

作者： Robert decker and Barbara decker

来源：畅想之星图书光盘版（南... 评论

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畅想之星图书光盘版（南通市图书馆镜像）

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World Journal of Emergency Medicine 2019年第2期10卷 119-121页

作者： Coralie galland-decker Vanessa Kraege Claudio Sartori Internal Medicine University HospitalLausanne-CHUV

Dear editor,Several types of tumors affect *** being rare,the Ewing sarcoma fami ly of tumors represents the second most common primary bone malignancy in children and *** Cushing syndrome i s a wel l-known condi t ion characterized by an ectopic secretion of ACTH(or CRH),and in turn of cortisol by tumor *** Cushing syndrome is however very rare in Ewing sarcoma,and to our knowledge,it was only described once.

关键词： Several types adolescents primary bone

Whole mount of adult ear skin as a model to study vascular malformations

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动物模型与实验医学(英文) 2023年第4期6卷 362-368页

作者： Brandee decker-Rockefeller Qingfen Li Kevin Pumiglia Biology Department Utica UniversityUticaNew YorkUSA Department of Regenerative and Cancer Cell Biology Albany Medical CollegeAlbanyNew YorkUSA

Background:genetic analysis in human patients has linked mutations in PIK3CA,the catalytic subunit of PI-3′Kinase,to sporadic incidences of vascular ***:We have developed a mouse model with inducible and endothelial-specific expression of PIK3CA H1047R,resulting in the development of vascular *** induction of this mutation in adult mice results in rapid lethality,limiting our ability to track and study these lesions;therefore,we developed a topical and local induction protocol using the active metabolite of tamoxifen,4OH-T,on the ear skin of ***:This approach allows us to successfully model the human disease in a mature and established vascular bed and track the development of vascular *** validate the utility of this model,we applied a topical rapamycin ointment,as rapamycin is therapeutically beneficial to patients in clinical *** found that the induced ear lesions showed significant attenuation after treatment,which was easily ***:These data collectively provide evidence of a new model to study vascular malformations in adult tissues,which should be particularly useful in environments lacking specialized small-animal imaging facilities.

关键词： animal disease models pathological angiogenesis PIK3CA rapamycin vascular malformations

Measurement of in-bore side loads and comparison to first maximum yaw

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Defence Technology（防务技术） 2016年第2期12卷 106-112页

作者： Donald E.CARLUCCI Ryan decker Julio VEgA Douglas RAY U.S.Army ARDEC RDAR-DSM Building 942nd FloorPicatinnyNJ 07806-5000USA U.S.Army ARDEC RDAR-EIQ-SB Building 62PicatinnyNJ 07806-5000USA

In-bore yaw of a projectile in a gun tube has been shown to result in range loss if the yaw is significant. An attempt was made to determine if relationships between in-bore yaw and projectile First Maximum Yaw(FMY) were observable. Experiments were conducted in which pressure transducers were mounted near the muzzle of a 155 mm cannon in three sets of four. Each set formed a cruciform pattern to obtain a differential pressure across the projectile. These data were then integrated to form a picture of what the overall pressure distribution was along the side of the projectile. The pressure distribution was used to determine a magnitude and direction of the overturning moment acting on the projectile. This moment and its resulting angular acceleration were then compared to the actual first maximum yaw observed in the test. The degree of correlation was examined using various statistical techniques. Overall uncertainty in the projectile dynamics was between 20% and 40% of the mean values of FMY.

关键词：偏航负载测量压力分布压力传感器发射动力学倾覆力矩角加速度统计技术

Metabolic activity of Streptococcus mutans biofilms and gene expression during exposure to xylitol and sucrose

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International Journal of Oral Science 2014年第4期6卷 195-204页

作者： Eva-Maria decker Christian Klein Dimitri Schwindt Christiane von Ohle Department for Pediatric and Operative Dentistry Periodontology and Endodontology Center of Dentistry Oral Medicine and Maxillofacial Surgery University of Tbingen Private Practice Zahngesundheit Waiblingen

The objective of the study was to analyse Streptococcus mutans biofilms grown under different dietary conditions by using multifaceted methodological approaches to gain deeper insight into the cariogenic impact of carbohydrates. S. mutans biofilms were generated during a period of 24 h in the following media： Schaedler broth as a control medium containing endogenous glucose, Schaedler broth with an additional 5% sucrose, and Schaedler broth supplemented with 1% xylitol. The confocal laser scanning microscopy（CLSM）-based analyses of the microbial vitality, respiratory activity（5-cyano-2,3-ditolyl tetrazolium chloride, CTC） and production of extracellular polysaccharides（EPS） were performed separately in the inner, middle and outer biofilm layers. In addition to the microbiological sample testing, the glucose/sucrose consumption of the biofilm bacteria was quantified, and the expression of glucosyltransferases and other biofilm-associated genes was investigated. Xylitol exposure did not inhibit the viability of S. mutans biofilms, as monitored by the following experimental parameters： culture growth, vitality, CTC activity and EPS production. However,xylitol exposure caused a difference in gene expression compared to the control. gtf C was upregulated only in the presence of *** xylitol exposure, gtf B was upregulated by a factor of 6, while under sucrose exposure, it was upregulated by a factor of *** with glucose and xylitol, sucrose increased cell vitality in all biofilm layers. In all nutrient media, the intrinsic glucose was almost completely consumed by the cells of the S. mutans biofilm within 24 h. After 24 h of biofilm formation, the multiparametric measurements showed that xylitol in the presence of glucose caused predominantly genotypic differences but did not induce metabolic differences compared to the control. Thus, the availability of dietary carbohydrates in either a pure or combined form seems to affect the cariogenic potential of

关键词： biofilms 5-cyano-2 3-ditolyl tetrazolium chloride extracellular polysaccharides gene expression Streptococcus mutans sucrose viability xylitol

PerformanceVis:Visual analytics of student performance data from an introductory chemistry course

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Visual Informatics 2019年第4期3卷 166-176页

作者： Haozhang Deng Xuemeng Wang Zhiyi guo Ashley decker Xiaojing Duan Chaoli Wang g.Alex Ambrose Kevin Abbott University of Rochester RochesterNY 14627United States Fudan University Shanghai 200433China University of Notre Dame Notre DameIN 46556United States

We present PerformanceVis,a visual analytics tool for analyzing student admission and course performance data and investigating homework and exam question *** a university-wide introductory chemistry course with nearly 1000 student enrollment,we consider the requirements and needs of students,instructors,and administrators in the design of *** study the correlation between question items from assignments and exams,employ machine learning techniques for student grade prediction,and develop an interface for interactive exploration of student course performance *** includes four main views(overall exam grade pathway,detailed exam grade pathway,detailed exam item analysis,and overall exam&homework analysis)which are dynamically linked together for user interaction and *** demonstrate the effectiveness of PerformanceVis through case studies along with an ad-hoc expert ***,we conclude this work by pointing out future work in this direction of learning analytics research.

关键词： Student performance Item analysis grade prediction Learning analytics Knowledge discovery