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Quantifying Kinematic Tremor in an NGLY1-Deficient Individual: A Case Study

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Case Reports in Clinical Medicine 2024年第1期13卷 25-36页

作者： Brock Futrell Christopher Alexander Malaya Dacia Martinez Diaz Christian Alfaro Hannah elizabeth Gustafson Subhalakshmi Chandrasekaran Rhea Mohan Phatak Bernhard Suter Charles Shannon Layne Department of Health and Human Performance University of Houston Houston USA Center for Neuromotor and Biomechanics Research University of Houston Houston USA Department of Computer Science and Engineering Texas A&M University College Station USA Blue Bird Circle Rett Center Texas Children’s Hospital Houston USA Baylor College of Medicine Houston USA Center for NeuroEngineering and Cognitive Science University of Houston Houston USA

NGLY1 Deficiency is an autosomal recessive congenital disorder that has been identified in less than 100 individuals. Most individuals with NGLY1 Deficiency display hyperkinetic movement disorders, including choreiform, athetoid, dystonic myoclonic, dyskinetic, and dysmetric movements. Developing a consistent and concise consensus on the classification and evaluation of tremors is essential to forward the research and treatment of tremors. It has also been reported that some individuals with NGLY1 Deficiency demonstrate tremor, but such tremor has never been formally investigated. The primary objective of this study is to determine if an individual with NGLY1 Deficiency demonstrates an identifiable tremor during a series of arm movements and, if so, describe the frequency and power characteristics of that tremor. Arm movement kinematics were obtained using a 16-camera Vicon system, and time series trajectory waveforms for three planes of a marker placed on the hand were developed. Custom MATLAB scripts were utilized to compute Fast Fourier Transformations of the data within the identified waveform segments. A mean frequency of 2.30 Hz (SD = 1.05) with a mean power of 5.02 |P1(f)| (SD = 4.63) suggests that our participant’s kinematic data did display a persistent tremor in both hands across all tasks and movement planes. Analyses of the reaching hand and the non-reaching hand suggest the participant displayed an action tremor in both postural and intention (kinetic) tremors. Future directions should include assessing additional individuals with NGLY1 Deficiency to determine if the tremor is a distinguishable disorder behavior. Additionally, evaluating other anatomical sites, such as the elbow, head, and lower limbs, would provide further insights into the characteristics of this tremor.

关键词： Tremor NGLY Fast Fourier Transformation Reaching Hyperkinetic Movement

Plasticity as panacea？ Nerves, hormones, and the currencies of trade-offs

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Current Zoology 2015年第2期61卷 251-264页

作者： elizabeth BASTIAANS elizabeth SWANGER Department of Ecology Evolution and Behavior University of Minnesota MN 55108 USA

Phenotypic plasticity is nearly universal among organisms, and evidence indicates that plasticity can exhibit additive genetic variation and respond to selection. These findings have important implications for our understanding of how plasticity may be constrained and how its mechanistic structure may affect its evolution. Many life history trade-offs may be conceptua- lized as plastic traits, with individuals varying in their position along trade-off axes due to genetic differences, developmental plasticity, or short-term plasticity occurring throughout an individual＇s lifetime. Behavioral plasticity is key to understanding when organisms are likely to encounter trade-offs, whether those trade-offs can be mitigated, and how the tradc-offs affect the ecology and evolution of populations. In this review, we discuss hormonal and neural mechanisms that may influence how plastic behavioral traits are expressed and evolve. We also outline a classification of life history trade-offs and their mechanistic bases and discuss the currencies most likely to mediate each category of trade-off and bow they are tied to the mechanisms by which animals express their behaviors.

关键词： Phenotypic plasticity Life history trade-offs Hormones Neural tissue

The effectiveness of eyespots and masquerade in protecting artificial prey across ontogenetic and seasonal shifts

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Current Zoology 2022年第4期68卷 451-458页

作者： elizabeth G Postema Department of Entomology and Nematology University of CaliforniaDavisCA 95616USA

When constraints on antipredator coloration shift over the course of development,it can be advantageous for animals to adopt different color strategies for each life *** caterpillars in the genus Papilio exhibit unique ontogenetic color sequences:for example,early instars that masquerade as bird feces,with later instars possessing eyespots.I hypothesize that larvae abandon feces masquerade in favor of eyespots due to ontogenetic changes in signaler *** ontogenetic pattern also occurs within broader seasonal shifts in background color and predator activity.I conducted predation experiments with artificial prey to determine how potential signaling constraints(specifically size and season)shape predation risk,and consequently the expression of ontogenetic color change in Papilio ***,both predation and background greenness declined significantly from July to September,though there was little evidence that these patterns impacted the effectiveness of either color *** size and color strongly affected the attack rate of avian predators:attacks increased with prey size regardless of color,and eyespotted prey were attacked more than masquerading prey *** results may reflect a key size-mediated tradeoff between conspicuousness and intimidation in eyespotted prey,and raise questions about how interwoven aspects of behavior and signal environment might maintain the prevalence of large,eyespotted larvae in nature.

关键词： ontogenetic color change eyespots masquerade predator-prey dynamics season

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PET/MRI改善癌症患儿管理

中华核医学与分子影像杂志 2024年第2期44卷 124-128页

作者： Lucia Baratto K.elizabeth Hawk Lisa States Jing Qi Sergios Gatidis Louise Kiru Heike E.Daldrup-Link 陈素芸(译) 王辉(审校) Department of Radiology Stanford UniversityStanfordCalifornia Department of Radiology Children′s Hospital of PhiladelphiaPhiladelphiaPennsylvania Department of Radiology Children′s WisconsinMilwaukeeWisconsin Department of Diagnostic and Interventional Radiology University Hospital TübingenTübingenGermany Department of Pediatrics Stanford UniversityStanfordCalifornia 上海交通大学医学院附属新华医院核医学科上海200092

一体化PET/MRI通过一站式成像提供肿瘤解剖学及功能评估,且与^(18)F-FDG PET/CT相比其辐射暴露量减少80%,因此对癌症患儿的分期与再分期具有显著临床价值。该文回顾了^(18)F-FDG PET/MRI在小儿肿瘤中的临床应用,尤其关注PET/MRI在患儿... 详细信息

一体化PET/MRI通过一站式成像提供肿瘤解剖学及功能评估,且与^(18)F-FDG PET/CT相比其辐射暴露量减少80%,因此对癌症患儿的分期与再分期具有显著临床价值。该文回顾了^(18)F-FDG PET/MRI在小儿肿瘤中的临床应用,尤其关注PET/MRI在患儿管理中的价值。来自4个不同机构的PET/MRI应用先行者分享了他们对PET/MRI技术在癌症患儿评估中特殊优势的见解。文章作者讨论了全身PET/MRI在评估某些解剖区域(如软组织和骨髓)中的价值以及儿童特殊的PET/MRI影像学特征,并强调了全身PET/MRI如何通过在不失去诊断准确性的同时减少所需放射学检查次数(因此减少辐射暴露),改善淋巴瘤、肉瘤和神经纤维瘤病患儿的临床管理。文章论述了PET/MRI如何帮助区分恶性肿瘤与感染性或炎性疾病;另外,简要探讨了PET/MRI用于接受免疫治疗的患儿疗效评估以及对不同诊疗一体化药物评估的未来研究方向。从儿童应用中所获得的经验也可拓展至成人患者的评估中。

关键词：肿瘤学儿科学 PET/MRI 癌症儿科肿瘤学

ntegrated approach to winery waste： waste generation and data consolidation

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Frontiers of Environmental Science & Engineering 2016年第1期10卷 168-176页

作者： Margarida OLIVEIRA elizabeth DUARTE Instituto Superior de Agronomia (ISA) University of Lisbon 1349-017 Lisboa Portugal Escola Superior Agrairia de Santarem (ESAS) Instituto Superior Politecnico de Santarem 2001-904 Santarem Portugal

The winemaking process involves the genera- tion of a significant amount of waste and wastewater. These residues should be addressed for recycling or treatment before being returned to environment. As each winery is unique in waste generation and disposal, plans for environmentally friendly waste management are not universal and should be tested for their effectiveness. In this study, a diagnostic was made during three years, in different wineries, throughout Portugal, in order to quantify and characterize the waste and the wastewater produced. The results showed that solid waste and wastewater are mainly produced during the harvest period, corresponding to 74% and 87%, respectively. One ton of processed grape approximately produce 0.13 t marc, 0.06 t lees, 0.03 t of stalks and 1.65 m3 of wastewater. No significant differences （P≤0.05） were observed for grape marc, lees and wastewater ratios, between years or wineries. With respect to the stalk ratio, there was no effect of year but the winery significantly affected this ratio （P ≤ 0.05）. During the study period the treated wastewater, since diluted, revealed suitable characteristics for irrigation representing an additional source of water. In this regard, the data acquisition and consolidation ensure the transfer of information and experience which constitute an essential step in a support decision tool design.

关键词： waste management wastewater reuse winerywastewater

淮北煤田中高阶煤储层物性表征及深部有利区预测研究

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非常规油气 2024年

作者：侯海海 elizabeth Cruz Pérez 陈泓圳胡博何倩黄乡琴辽宁工程技术大学矿业学院

为定量表征淮北煤田中高阶煤储层物性特征，采用工业分析、显微煤岩组分定量、低温氮吸附、高压压汞、核磁共振等实验手段，对淮北煤田7个煤样的物性特征进行了研究。结果表明，淮北煤田煤显微组分以镜质组为主，最大镜质组反射率变化... 详细信息

为定量表征淮北煤田中高阶煤储层物性特征，采用工业分析、显微煤岩组分定量、低温氮吸附、高压压汞、核磁共振等实验手段，对淮北煤田7个煤样的物性特征进行了研究。结果表明，淮北煤田煤显微组分以镜质组为主，最大镜质组反射率变化较大，在0.7%-2.5%之间，可能受燕山期郯庐和太行山岩浆活动的共同影响。该煤质具有中-高灰分、低-极低硫含量、低磷、中高热值的特点。从低阶煤到中阶煤，由于脱水作用较强，煤基质收缩，吸附孔隙和渗流孔隙体积减小，裂隙体积增大；从中阶煤到高阶煤，吸附孔具有明显的优势，表明其对甲烷的吸附能力增强。预测淮北煤田宿县矿区的南坪深部、桃园祁南深部、彭桥深部、朱仙庄北部，临涣矿区的任楼赵集深部、许疃深部，濉萧矿区的萧西深部为该区煤层气勘探的较有利区，

关键词：淮北煤田煤储层物性孔裂隙结构煤层气有利区优选

Crystal Structure of the MAP3K TAO2 Kinase Domain Bound by an Inhibitor Staurosporine

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Acta Biochimica et Biophysica Sinica 2006年第6期38卷 385-392页

作者： elizabeth J.GOLDSMITH USA TX 75390-9038 Department of Biochemistry University of Texas Southwestern Medical Center at Dallas

Mitogen-activated protein kinase (MAPK) signal transduction pathways are ubiquitous ineukaryotic cells,which transfer signals from the cell surface to the nucleus,controlling multiple *** are activated by MAPK kinases [MAP2Ks or MAP/extracellular signal-regulated kinase(ERK) kinases (MEK)],which in turn are activated by MAPK kinase kinases (MAP3Ks).TAO2 is a MAP3Klevel kinase that activates the MAP2Ks MEK3 and MEK6 to activate p38 *** p38 MAPKs arekey regulators of expression of inflammatory cytokines,they appear to be involved in human diseases suchas asthma and *** an upstream activator of p38s,TAO2 represents a potential drug *** we report the crystal structure of active TAO2 kinase domain in complex with staurosporine,a broad-range protein kinase inhibitor that inhibits TAO2 with an ICof 3 μ*** structure reveals that staurosporineoccupies the position where the adenosine of ATP binds in TAO2,and the binding of the inhibitor mimicsmany features of ATP *** polar and nonpolar interactions contribute to the *** induces conformational changes in TAO2 residues that surround the inhibitormolecule,but causes very limited global changes in the *** structure provides atomic details forTAO2-staurosporine interactions,and explains the relatively low potency of staurosporine against *** structure presented here should aid in the design of inhibitors specific to TAO2 and related kinases.

关键词： TAO2 MAP3K inhibitor staurosporine crystal structure

The Sendai Framework for Disaster Risk Reduction at Five:Lessons from the 2011 Great East Japan Earthquake and Tsunami

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International Journal of Disaster Risk Science 2020年第2期11卷 167-178页

作者： elizabeth Maly Anawat Suppasri International Research Institute of Disaster Science Tohoku UniversitySendai 980-8579.Japan

The Sendai Framework for Disaster Risk Reduction 2015–2030(SFDRR) represents an inclusive approach to disaster risk reduction, linked to development and recovery. Building on previous international guidelines, the SFDRR promotes practical and measurable outcomes for reducing disaster losses, including indicators to measure progress towards seven specific global *** in the context of the priorities and global targets of the SFDRR, the article considers the case of the Great East Japan Earthquake and Tsunami of 2011 to illustrate advances and limitations in pre-disaster tsunami hazard engineering and post-disaster recovery. With respect to the targets set out in the Sendai Framework related to risk reduction and tsunami engineering, especially Priority 1,understanding disaster risk, there have been significant advances in hazard assessment and systems for tsunami detection and warning. Although the SFDRR emphasizes actions in the recovery phase through Priority 4, enhancing disaster preparedness with an emphasis on the concept of build back better, on its own this does not function as a clear directive for guiding recovery. However, the overall people-centered and inclusive approach of the SFDRR, as well as the global targets and several sub-priorities, can be a useful way to shape good practices in recovery and evaluate recovery progress.

关键词： Build back better Disaster recovery Great East Japan Earthquake Sendai Framework Tsunami risk

Distribution of Panama’s narrow-range trees:are there hot-spots?

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Forest Ecosystems 2021年第4期8卷 801-809页

作者： elizabeth Tokarz Richard Condit Morton Arboretum 4100 Illinois Rte.53LisleIL 60532USA

Background:Tree species with narrow ranges are a conservation concern because heightened extinction risk accompanies their small *** risks for these species is challenging,however,especially in tropical flora where their sparse populations seldom appear in traditional plots and ***,we utilize instead large scale databases that combine tree records from many sources to test whether the narrow-range tree species of Panama are concentrated at certain elevations or in certain *** investigations have suggested that the Choco region of eastern Panama and the high mountains of western Panama may be potential hotspots of narrow-range tree ***:All individual records were collected from public databases,and the range size of each tree species found in Panama was estimated as a polygon enclosing all its *** with ranges<20,000 km^(2) were defined as narrow *** divided Panama into geographic regions and elevation zones and counted the number of individual records and the species richness in each,separating narrow-range species from all other ***:The proportion of narrow endemics peaked at elevations above 2000 m,reaching 17.2% of the species *** elevation<1500 m across the country,the proportion was 6-11%,except in the dry Pacific region,where it was 1.5%.Wet forests of the Caribbean coast had 8.4% narrow-range species,slightly higher than other *** total number of narrow endemics,however,peaked at mid-elevation,not high elevation,because total species richness was highest at ***:High elevation forests of west Panama had higher proportions of narrow endemic trees than low-elevation regions,supporting their hot-spot status,while dry lowland forests had the lowest *** supports the notion that montane forests of Central America should be a conservation ***,given generally higher diversity at low-to mid-elevation,lowlands are also import

关键词： Narrow endemic trees Biodiversity hot-spots Panama Rarefaction High elevations BIEN database Narrow-range