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Natural history of Usher type 2 with the c.2299delG mutation of USH2A in a large cohort

OPHTHALMIc GENETIcS 2022年第4期43卷 470-475页

作者： Meunier, Audrey Zanlonghi, Xavier Roux, Anne-Francoise Fils, Jean-Francois caspers, Laure Migeotte, Isabelle Abramowicz, Marc Meunier, Isabelle Univ Libre Bruxelles ULB Dept Ophthalmol Univ Hosp St Pierre Brussels Belgium Jules Verne Clin Ctr Competence Malad Rares Nantes France Univ Montpellier Montpellier Univ Hosp Lab Mol Genet Montpellier France Univ Montpellier Inst Neurosci Montpellier INSERM Montpellier France Ars Stat Nivelles Belgium Univ Libre Bruxelles Genet Dept IRIBHM Brussels Belgium Univ Geneva Fac Med Dept Genet Med & Dev Geneva Switzerland Univ Hosp Natl Ctr Rare Dis Maolya Sensgene Care Network Genet Sensory Dis Montpellier France

Background The c.2299delG mutation is prevalent and accounts for 24.5% USH2A pathogenic variants, with promising prospects for customized gene therapy. Materials and Methods We compared the ocular and auditory phenotypes in a retrospective cohort of 169 Usher type 2 patients, with and without the c.2299delG allele, including visual acuity, slit-lamp examination, optical coherence tomography, kinetic perimetry, and audiometric assessment to define the hearing disability. Statistical methods used were covariate balancing propensity score and adjusted survival curves log-rank test for the analysis of visual acuity. Results We compare 54 Usher patients (31%) carrying at least one c.2299delG allele to 109 patients without this variant. The mean ages at onset of night blindness (14 years) and onset of peripheral vision deficiency (24 years) were similar in both groups, as was the severity of hearing loss (p = 0.731), even in homozygotes (p = 0.136). Based on the covariate balancing propensity score, the c.2299delG carrier patients developed cataract and reached a BcVA of 20/63 earlier than patients without this mutation (mean age 36 versus 42 y.o.;and 52.2 versus 55.1 y.o., respectively). Using adjusted survival curves and a log-rank test based on inverse probability weighting, patients with the c.2299delG variant reach blindness (BcVA <20/400) at 42.3 years old instead of 79.8 years for other USH2A pathogenic variants. conclusions We conclude that c.2299delG is associated with a more severe phenotype of the Usher type 2, in homozygotes and in compound heterozygotes.

关键词： Usher retinitis pigmentosa c 2299delG USH2A propensity score

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Benefits of Using the Invertebrate Model Organism c. Elegans in an Inquiry-Based Laboratory Activity

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TEAcHING OF PSYcHOLOGY 2023年第1期50卷 26-31页

作者： Kallarackal, Angy J. Mt St Marys Univ Psychol Dept 16300 Old Emmitsburg Rd Emmitsburg MD 21217 USA

Background: The goals of laboratory experiences include developing knowledge base, research skills, and scientific communication abilities. Objective: The aim was to assess an inquiry-based laboratory activity using the model organism caenorhabditis elegans in relation to learning goals. Method: Students in a Biopsychology laboratory course worked in groups to test the effect of various drugs (e.g., nicotine, ethanol, fluoxetine, and melatonin) on c. elegans behavior. The activity included literature review, experimental design, and a final lab report. A cumulative final exam included a synaptic communication question related to the content of the activity. Results: Students showed better retention of laboratory-related content compared to other topics from the course, as demonstrated through performance on the final exam and were able to replicate previous research demonstrating effects of drug on locomotion. However, students did not improve writing ability compared to performance on a previous American Psychological Association style lab report. conclusion: This study demonstrates that using a student-designed, multi-week laboratory assignment in an undergraduate Biopsychology course supports the growth of psychology knowledge and the development of research skills. Teaching Implications: Instructors should consider using the described laboratory activity for biopsychology or behavioral neuroscience classes or consider similarly designed laboratory formats for other courses in Psychology.

关键词： drugs c elegans inquiry locomotion biopsychology neuroscience

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Extinction Panic: c. S. Lewis and Planetary Nihilism

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MODERN LANGUAGE QUARTERLY 2023年第1期84卷 27-51页

作者： Harper, Tyler Austin Bates Coll Environm Studies Lewiston ME 04240 USA

Today figures like Elon Musk and Jeff Bezos have become high-profile evangelists for private space exploration, arguing that interplanetary colonization is necessary to save human-ity from extinction. Although they may have the sheen of innovation, however, these ideas are not new. A century ago a coterie of British novelists, scientists, and social theorists writing dur-ing the interwar period became preoccupied with the possibility of human extinction and believed that such a fate might be avoided by taking human civilization to the stars. Watching these intellectual developments with a wary eye, a young c. S. Lewis was increasingly skeptical of both the "extinction panic" that gripped his contemporaries and the utilitarian and environmen-tally exploitative imagination of planetary conquest they championed. In response Lewis penned Out of the Silent Planet (1938), a novel that imagines three sentient species that have the means to prevent their own extinction but choose not to do so. Reading Lewis's novel as a critique of the rapacious ideologies that defined this strain of interwar speculation, this article suggests that the novel models how the humanities might effectively respond to the extinction panic and cosmic adventurism that grip our own imperiled twenty-first century.

关键词： extinction existential risk c S Lewis interwar science fiction

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Involvement of Endoplasmic Reticulum Stress-Mediated Activation of c/EBP Homologous Protein in Aortic Regurgitation-Induced cardiac Remodeling in Mice

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JOURNAL OF cARDIOVAScULAR TRANSLATIONAL RESEARcH 2022年第2期15卷 340-349页

作者： Wang, Xingxu Wei, Wei Wu, Jian Kang, Le Wu, Shuangquan Li, Jiming Shen, Yunli You, Jieyun Ye, Yong Zhang, Qi Zou, Yunzeng Tongji Univ Shanghai East Hosp Dept Cardiol Sch Med Shanghai 200120 Peoples R China Fudan Univ Zhongshan Hosp Shanghai Inst Cardiovasc Dis Shanghai 200032 Peoples R China Fudan Univ Inst Biomed Sci Shanghai 200032 Peoples R China Jiaozhoushi Peoples Hosp Dept Cardiol Qingdao 266300 Peoples R China

Aortic regurgitation (AR) is a volume overload disease causing eccentric left ventricular (LV) hypertrophy and eventually heart failure. There is currently no approved drug to treat patients with AR. Endoplasmic reticulum (ER) stress and ER stress-mediated apoptosis is involved in many cardiovascular diseases, but whether they also participate in AR-induced heart failure is still elusive. In this study, we found ER stress activation in myocardial samples from patients with AR. With a unique murine model of AR which induced eccentric cardiac hypertrophy and heart failure, we also found aggravation of cardiac ER stress and apoptosis, as evidenced by a reduction of Bcl-2/Bax ratio and an increase of caspase-3 cleavage. We then examined the signaling effectors involved in ER-initiated apoptosis and found volume overload specifically activated c/EBP homologous protein (cHOP), but not caspase-12 or Jun N-terminal kinase (JNK). Interestingly, tauroursodeoxycholic acid (TUDcA), an ER stress inhibitor, improved cardiac function, and suppressed ER stress, apoptosis, and cHOP. Furthermore, genetic knockdown of cHOP inhibited cardiac Bcl-2/Bax ratio reduction and caspase-3 activation and rescued cardiac dysfunction. In summary, our findings suggest that ER stress-cHOP signaling is involved in the development of volume overload cardiac hypertrophy induced by AR through promoting cardiomyocytes apoptosis and provide a previously unrecognized target in heart failure induced by volume overload.

关键词： Apoptosis Endoplasmic reticulum stress c EBP homologous protein Aortic regurgitation Volume overload

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Larvae and fry culture of white cachama (Piaractus orinoquensis) in biofloc systems at different carbon/nitrogen (c/N) ratios

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AQUAcULTURE RESEARcH 2022年第3期53卷 799-809页

作者： Lasso, Luis Felipe collazos castellanos, Jose Alfredo Arias Fukura, Mitsuaki Ueno carrasco, Sandra clemencia Pardo Arana, Luis Alejandro Vinatea Univ Llanos Fac Ciencias Agr & Recursos Natur FCARN Inst Agr Grp Invest IALL Villavicencio Colombia Univ Nacl Colombia Fac Ciencias Agr Dept Prod Anim Sede Medellin Colombia Univ Fed Santa Catarina Dept Aquicultura Lab Camaroes Marinhos Florianopolis SC Brazil

This work evaluated the effects of different carbon/nitrogen (c/N) ratios in the larvae and fry culture of Piaractus orinoquensis in biofloc system on the water quality, proximate analysis and growth performance. The experiment was conducted in two stages: (a) larval stage from 0 to 12 days post stocking (DPS) and (b) fry stage from 13 to 24 DPS, at a density of 10 larvae/L. Three groups of c/N ratios were evaluated as follows: T1: 10/1 ratio, T2: 15/1 ratio and T3: 20/1 ratio, each treatment with three replicates. Both in the larval and fry culture, total ammonia nitrogen (TAN) and nitrite were higher in the T2 and T3 treatments, while the nitrogen removal in T1 treatment was more efficient, resulting less TAN, nitrite and higher nitrate. However, the water quality parameters in all treatments were within the appropriate ranges for fish culture in biofloc. T2 treatment was nutritionally better, presenting higher levels of protein, lipids and energy. In both stages, P. orinoquensis survival and final weight were significantly higher in the T2 treatment. In conclusion, P. orinoquensis larvae and fry culture in biofloc is viable, improving the survival and growth at a c/N ratio of 15/1.

关键词： biofloc technology c N ratio larviculture protein survival white cachama

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Inhibitors of Jumonji c domain-containing histone lysine demethylases overcome cisplatin and paclitaxel resistance in non-small cell lung cancer through APc/cdh1-dependent degradation of ctIP and PAF15

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cANcER BIOLOGY & THERAPY 2022年第1期23卷 65-75页

作者： Duan, Lei Perez, Ricardo E. calhoun, Sarah Maki, carl G. Rush Univ Med Ctr Dept Cell & Mol Med 600 S Paulina AveAcFAC 507 Chicago IL 60612 USA Northwestern Univ Robert H Lurie Comprehens Canc Ctr Chicago IL 60611 USA

The Jumonji c domain-containing family of histone lysine demethylases (Jumonji KDMs) have emerged as promising cancer therapy targets. These enzymes remove methyl groups from various histone lysines and, in turn, regulate processes including chromatin compaction, gene transcription, and DNA repair. Small molecule inhibitors of Jumonji KDMs have shown promise in preclinical studies against non-small cell lung cancer (NScLc) and other cancers. However, how these inhibitors influence cancer therapy responses and/or DNA repair is incompletely understood. In this study, we established cell line and PDX tumor model systems of cisplatin and paclitaxel-resistant NScLc. We showed that resistant cells and tumors express high levels of Jumonji-KDMs. Knockdown of individual KDMs or treatment with a pan-Jumonji KDM inhibitor sensitized the cells and tumors to cisplatin and paclitaxel and blocked NScLc in vivo tumor growth. Mechanistically, we found inhibition of Jumonji-KDMs triggers APc/cdh1-dependent degradation of ctIP and PAF15, two DNA repair proteins that promote repair of cisplatin and paclitaxel-induced DNA lesions. Knockdown of ctIP and PAF15 sensitized resistant cells to cisplatin, indicating their degradation when Jumonji KDMs are inhibited contributes to cisplatin sensitivity. Our results support the idea that Jumonji-KDMs are a targetable barrier to effective therapy responses in NScLc. Inhibition of Jumonji KDMs increases therapy (cisplatin/paclitaxel) sensitivity in NScLc cells, at least in part, by promoting APc/cdh1-dependent degradation of ctIP and PAF15.

关键词： Histone demethylase APc c ctIP PAF15 cisplatin NScLc

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Plant c:N ratio predicts community composition changes under N addition in an alpine meadow

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JOURNAL OF EcOLOGY 2022年第3期111卷 589-600页

作者： Peng, Jinlong Ma, Fangfang Tian, Dashuan Quan, Quan chen, chen Liao, Jiaqiang Zhou, Qingping Niu, Shuli Chinese Acad Sci Inst Geog Sci & Nat Resources Res Key Lab Ecosyst Network Observat & Modeling Beijing Peoples R China Univ Chinese Acad Sci Coll Resources & Environm Beijing Peoples R China Southwest Univ Nationalities Inst Qinghai Tibetan Plateau Chengdu Peoples R China

Plant functional traits play important roles in determining plant responses to environmental change and further shaping community composition, but the role of plant stoichiometry remains poorly understood in regulating community responses to continually increased nitrogen (N) deposition. In this paper, we used a 5-year manipulative experiment with six N addition rates (N-rate) to explore how the N response of different plant functional groups including forbs, grasses, sedges and legumes links to above-ground plant carbon-to-N ratio (c:N, related to N use strategies) in an alpine meadow. We found that plant c:N explained 45.8% and 42.6% of N response variation among functional groups in plant above-ground net primary productivity (ANPP) and abundance. Over increasing N-rate, functional groups with higher c:N (grasses and sedges) tended to grow better and have greater abundance, yet those with lower c:N (forbs and legumes) were at risk of loss, subsequently mitigating the N-caused decline in community-level plant c:N. This was mainly due to that functional groups with higher c:N not only possessed higher N use efficiency but also had greater competitiveness for above-ground light due to higher plant height. In addition, we also found that plant c:N differences among functional groups remained relatively constant over N-rate, suggesting that the ability of this trait to indicate functional group-specific N responses was stable. Synthesis. Our results show that the N responses of different herbaceous plants can be well explained by plant c:N. Thus, we suggest the prediction of plant community composition changes under N deposition would be greatly improved by considering this easily measured stoichiometric trait.

关键词： alpine meadow c N ratio ecological stoichiometry functional trait N deposition plant community composition productivity

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Effects of Fe doping on the electrochemical performance of LiV1-xFexPO4F/c (x=0, 0.01, 0.02, 0.04) cathode materials for lithium-ion batteries

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JOURNAL OF THE cHINESE cHEMIcAL SOcIETY 2022年第5期69卷 786-793页

作者： Zhang, Xinyi Di, Yuli Jiang, Fayou Jiang, Ao Deng, Lin Li, Taishan Dong, Qi chen, Shanhua Chengdu Univ Technol Coll Mat & Chem & Chem Engn Chengdu 610059 Peoples R China Xichang Univ Sch Sci Xichang Peoples R China Panzhuhua Univ Sch Biol & Chem Engn Panzhihua Peoples R China

The Fe-doped LiVPO4F/c cathode material was synthesized by a one-step solid-state reaction method. The effects of Fe doping content on the structure and electrochemical performance of LiVPO4F/c were investigated. X-ray diffraction (XRD) analyses demonstrated that the appropriate addition of Fe3+ did not destroy the lattice structure of LiVPO4F/c. Scanning electron microscopy (SEM) showed that the surface of LiVPO4F/c is covered by a carbon film. The decreased grain size of the Fe-doped LiVPO4F/c could shorten the ion transport path and thus improve the migration rate of lithium ions. X-ray photoelectron spectroscopy (XPS) showed that the Fe ion exists in the Fe-doped LiVPO4F/c as Fe3+ ion. At the discharge rate of 1 c, the initial discharge capacity and capacity retention rate of the LiV0.98Fe0.02PO4F/c after 500 cycles were 123.5 mAh/g and 88.6%, respectively.

关键词： electrochemical performance Fe dopant LiVPO4F c one-step solid-state reaction

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Asymmetric organelle positioning during epithelial polarization of c. elegans intestinal cells

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DEVELOPMENTAL BIOLOGY 2022年第0期481卷 75-94页

作者： Brandt, James N. Voss, Laura Rambo, Fiona M. Nicholson, Katelyn Thein, Jackson R. Fairchild, Lydia Seabrook, Laurence Lewis, Danielia Guevara-Hernandez, Lali White, Matthew L. Sax, Luca Eichten, Victoria Harper, Logan Hermann, Greg J. Lewis & Clark Coll Dept Biol Portland OR 97219 USA

While the epithelial cell cortex displays profound asymmetries in protein distribution and morphology along the apico-basal axis, the extent to which the cytoplasm is similarly polarized within epithelial cells remains relatively unexplored. We show that cytoplasmic organelles within c. elegans embryonic intestinal cells develop extensive apico-basal polarity at the time they establish cortical asymmetry. Nuclei and conventional endosomes, including early endosomes, late endosomes, and lysosomes, become polarized apically. Lysosome-related gut granules, yolk platelets, and lipid droplets become basally enriched. Removal of par-3 activity does not disrupt organelle positioning, indicating that cytoplasmic apico-basal asymmetry is independent of the PAR polarity pathway. Blocking the apical migration of nuclei leads to the apical positioning of gut granules and yolk platelets, whereas the asymmetric localization of conventional endosomes and lipid droplets is unaltered. This suggests that nuclear positioning organizes some, but not all, cytoplasmic asymmetries in this cell type. We show that gut granules become apically enriched when WHT-2 and WHT-7 function is disrupted, identifying a novel role for ABcG transporters in gut granule positioning during epithelial polarization. Analysis of WHT-2 and WHT-7 ATPase mutants is consistent with a WHT-2/WHT-7 heterodimer acting as a transporter in gut granule positioning. In wht2(-) mutants, the polarized distribution of other organelles is not altered and gut granules do not take on characteristics of conventional endosomes that could have explained their apical mispositioning. During epithelial polarization wht-2(-) gut granules exhibit a loss of the Rab32/38 family member GLO-1 and ectopic expression of GLO-1 is sufficient to rescue the basal positioning of wht-2(-) and wht-7(-) gut granules. Furthermore, depletion of GLO-1 causes the mislocalization of the endolysosomal RAB-7 to gut granules and RAB-7 drives the apical mis

关键词： Epithelial polarization Organelle positioning ABc transporter Rab GTPase c elegans

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Latrophilin-1 drives neuron morphogenesis and shapes chemo- and mechanosensation-dependent behavior in c. elegans via a trans function

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BIOcHEMIcAL AND BIOPHYSIcAL RESEARcH cOMMUNIcATIONS 2022年 589卷 152-158页

作者： Matus, Daniel Post, Willem Berend Horn, Susanne Schoeneberg, Torsten Proemel, Simone Univ Leipzig Med Fac Rudolf Schonheimer Inst Biochem D-04103 Leipzig Germany Heinrich Heine Univ Dusseldorf Inst Cell Biol Dept Biol D-40225 Dusseldorf Germany

Latrophilins are highly conserved Adhesion GPcRs playing essential roles in the mammalian nervous system and are associated with severe neurological disorders. Recently, it has been shown that murine Latrophilins mediate classical G-protein signals to drive synaptogenesis. However, there is evidence that Latrophilins in the nematode caenorhabditis elegans can also function independently of their seventransmembrane domain and c terminus (trans function). Here, we show that Latrophilin-1 acts in trans to mediate morphogenesis of sensory structures in the c. elegans nervous system. This trans function is physiologically relevant in copulation behavior. Detailed expression and RNA-Seq analyses revealed specific LAT-1-positive neurons and first insights into the genetic network that is modulated by the receptor function. We conclude that 7TM-independent functions of Latrophilins are essential for neuronal physiology, possibly complementing canonical functions via G protein-mediated signaling. (c) 2021 Elsevier Inc. All rights reserved.

关键词： Adhesion GPcR Latrophilin Neuron morphogenesis copulation behavior trans function c elegans

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