Miracle-dz/UniFAR-FTUs huggingface dataset:
Temporal Phenotypic Changes in Huntington’s Disease Models for Preclinical Studies

Background: Mouse models bearing genetic disease mutations are instrumental in the development of therapies for genetic disorders. Huntington’s disease (HD) is a late-onset lethal dominant genetic disorder due to a CAG repeat within exon 1 of the Huntingtin (Htt) gene. Several mice were developed to model HD through the expression of a transgenic fragment (exon 1 of the human HTT), the knock-in mutation of the CAG repeat in the context of the mouse Htt gene, or the full-length HTT human gene. The different mouse models present distinct onset, symptoms, and progression of the disease. Objective: The objective of this study is to advise on the best behavioral tests to assess disease progression in three HD mouse models. Methods: We tested N171-82Q transgenic mice, zQ175 knock-in mice, and BACHD full-length mice in a comprehensive behavior test battery in early, mid-, and late disease stages. Results: We contrast and compare the models and the emerging phenotypes with the available literature. These results suggest the most effective behavioral tests and appropriate sample sizes to detect treatment efficacy in each model at the different ages. We provide options for early detection of motor deficits while minimizing testing time and training. Conclusion: This information will inform researchers in the HD field as to which mouse model, tests and sample sizes can accurately and sensitively detect treatment efficacy in preclinical HD research.
Web Journalism: Practice and Promise of a New Medium

From the Publisher: Journalism on the Web explores the current practices and future possibilities of Web journalism and examines the characteristics of the Web that distinguish it from traditional media. The author guides students through discussion of the traditional practices of journalism, such as reporting, editing, photojournalism, and design, while showing how the distinguishing features of the Web -- capacity, immediacy, flexibility, permanency, and interactivity - offer new storytelling possibilities. The traditional principles of journalism, particularly journalistic writing that emphasizes accuracy, clarity, precision and efficiency, are emphasized throughout the text. Features The first three chapters offer an in-depth examination of the Web as an individual news medium, taking students beyond the idea that Web news is simply a newspaper on screen.An inside look at MSNBC provides students with an idea of what it's like to be inside a 24-hour Web news organization (Ch. 13). Three chapters (4, 5, and 6) on lateral thinking ask students to think beyond the traditional narrative storytelling forms of the inverted pyramid and present a variety of forms and structures to present information. "Cool Ideas" sidebars, located throughout the book, offer short descriptions of innovative ideas of Web journalism, to stimulate creative thinking.Web site references at the end of each chapter provide professors with the best sites available for keeping up with advances in Web journalism.
THE EFFECTS OF NOISE CALCULATION METHODS ON ANNOYANCE AND HEALTH ASSOCIATION ESTIMATION

Although this is an excellent way to test the accuracy of the sound propagation and source model in terms of physical intensity, there are further aspects of the sound climate to consider which determine the reaction of humans to the actually perceived and psycho-psychologically processed sound input. Without evaluation of the various acoustic models in the framework of the actual noise – health relations something important is missing.
GLYCINE-RICH RNA-BINDING PROTEIN 7 potentiates effector-triggered immunity through an RNA recognition motif

Abstract The activity of intracellular plant nucleotide-binding leucine-rich repeat (NB-LRR) immune receptors is fine-tuned by interactions between the receptors and their partners. Identifying NB-LRR interacting proteins is therefore crucial to advance our understanding of how these receptors function. A co-immunoprecipitation/mass spectrometry screening was performed in Nicotiana benthamiana to identify host proteins associated with the resistance protein Gpa2, a CC-NB-LRR immune receptor conferring resistance against the potato cyst nematode Globodera pallida. A combination of biochemical, cellular, and functional assays was used to assess the role of a candidate interactor in defense. A N. benthamiana homolog of the GLYCINE-RICH RNA-BINDING PROTEIN7 (NbGRP7) protein was prioritized as a Gpa2-interacting protein for further investigations. NbGRP7 also associates in planta with the homologous Rx1 receptor, which confers immunity to Potato Virus X. We show that NbGRP7 positively regulates extreme resistance by Rx1 and cell death by Gpa2. Mutating the NbGRP7 RNA recognition motif (RRM) compromises its role in Rx1-mediated defense. Strikingly, ectopic NbGRP7 expression is likely to impact the steady-state levels of Rx1, which relies on an intact RRM. Our findings illustrate that NbGRP7 is a pro-immune component in effector-triggered immunity by regulating Gpa2/Rx1 function at a posttranscriptional level.
Cytokine Hemoadsorption During Cardiac Surgery Versus Standard Surgical Care for Infective Endocarditis (REMOVE): Results From a Multicenter Randomized Controlled Trial

Background: Cardiac surgery often represents the only treatment option in patients with infective endocarditis (IE). However, IE surgery may lead to a sudden release of inflammatory mediators, which is associated with postoperative organ dysfunction. We investigated the effect of hemoadsorption during IE surgery on postoperative organ dysfunction. Methods: This multicenter, randomized, nonblinded, controlled trial assigned patients undergoing cardiac surgery for IE to hemoadsorption (integration of CytoSorb to cardiopulmonary bypass) or control. The primary outcome (change in sequential organ failure assessment score [ΔSOFA]) was defined as the difference between the mean total postoperative SOFA score, calculated maximally to the 9th postoperative day, and the basal SOFA score. The analysis was by modified intention to treat. A predefined intergroup comparison was performed using a linear mixed model for ΔSOFA including surgeon and baseline SOFA score as fixed effect covariates and with the surgical center as random effect. The SOFA score assesses dysfunction in 6 organ systems, each scored from 0 to 4. Higher scores indicate worsening dysfunction. Secondary outcomes were 30-day mortality, duration of mechanical ventilation, and vasopressor and renal replacement therapy. Cytokines were measured in the first 50 patients. Results: Between January 17, 2018, and January 31, 2020, a total of 288 patients were randomly assigned to hemoadsorption (n=142) or control (n=146). Four patients in the hemoadsorption and 2 in the control group were excluded because they did not undergo surgery. The primary outcome, ΔSOFA, did not differ between the hemoadsorption and the control group (1.79±3.75 and 1.93±3.53, respectively; 95% CI, –1.30 to 0.83; P=0.6766). Mortality at 30 days (21% hemoadsorption versus 22% control; P=0.782), duration of mechanical ventilation, and vasopressor and renal replacement therapy did not differ between groups. Levels of interleukin-1β and interleukin-18 at the end of integration of hemoadsorption to cardiopulmonary bypass were significantly lower in the hemoadsorption than in the control group. Conclusions: This randomized trial failed to demonstrate a reduction in postoperative organ dysfunction through intraoperative hemoadsorption in patients undergoing cardiac surgery for IE. Although hemoadsorption reduced plasma cytokines at the end of cardiopulmonary bypass, there was no difference in any of the clinically relevant outcome measures. Registration: URL: https://www.clinicaltrials. gov; Unique identifier: NCT03266302.
Comparison of All Solid Cancer Mortality and Incidence Dose-Response in the Life Span Study of Atomic Bomb Survivors, 1958–2009

Recent analysis of all solid cancer incidence (1958–2009) in the Life Span Study (LSS) revealed evidence of upward curvature in the radiation dose response among males but not females. Upward curvature in sex-averaged excess relative risk (ERR) for all solid cancer mortality (1950–2003) was also observed in the 0–2 Gy dose range. As reasons for non-linearity in the LSS are not completely understood, we conducted dose-response analyses for all solid cancer mortality and incidence applying similar methods [1958–2009 follow-up, DS02R1 doses, including subjects not-in-city (NIC) at the time of the bombing] and statistical models. Incident cancers were ascertained from Hiroshima and Nagasaki cancer registries, while cause of death was ascertained from death certificates throughout Japan. The study included 105,444 LSS subjects who were alive and not known to have cancer before January 1, 1958 (80,205 with dose estimates and 25,239 NIC subjects). Between 1958 and 2009, there were 3.1 million person-years (PY) and 22,538 solid cancers for incidence analysis and 3.8 million PY and 15,419 solid cancer deaths for mortality analysis. We fitted sex-specific ERR models adjusted for smoking to both types of data. Over the entire range of doses, solid cancer mortality dose-response exhibited a borderline significant upward curvature among males (P = 0.062) and significant upward curvature among females (P = 0.010); for solid cancer incidence, as before, we found a significant upward curvature among males (P = 0.001) but not among females (P = 0.624). The sex difference in magnitude of dose-response curvature was statistically significant for cancer incidence (P = 0.017) but not for cancer mortality (P = 0.781). The results of analyses in the 0–2 Gy range and restricted lower dose ranges generally supported inferences made about the sex-specific dose-response shape over the entire range of doses for each outcome. Patterns of sex-specific curvature by calendar period (1958–1987 vs. 1988–2009) and age at exposure (0–19 vs. 20–83) varied between mortality and incidence data, particularly among females, although for each outcome there was an indication of curvature among 0–19-year-old male survivors in both calendar periods and among 0–19-year-old female survivors in the recent period. Collectively, our findings indicate that the upward curvature in all solid cancer dose response in the LSS is neither specific to males nor to incidence data; its evidence appears to depend on the composition of sites comprising all solid cancer group and age at exposure or time. Further follow up and site-specific analyses of cancer mortality and incidence will be important to confirm the emerging trend in dose-response curvature among young survivors and unveil the contributing factors and sites.
Phenotypic spectrum of BLM‐ and RMI1‐related Bloom syndrome

Bloom syndrome (BS) is an autosomal recessive disorder with characteristic clinical features of primary microcephaly, growth deficiency, cancer predisposition, and immunodeficiency. Here, we report the clinical and molecular findings of eight patients from six families diagnosed with BS. We identified causative pathogenic variants in all families including three different variants in BLM and one variant in RMI1. The homozygous c. 581_582delTT;p.Phe194* and c.3164G>C;p.Cys1055Ser variants in BLM have already been reported in BS patients, while the c. 572_573delGA;p.Arg191Lysfs*4 variant is novel. Additionally, we present the detailed clinical characteristics of two cases with BS in which we previously identified the biallelic loss‐of‐function variant c. 1255_1259delAAGAA;p.Lys419Leufs*5 in RMI1. All BS patients had primary microcephaly, intrauterine growth delay, and short stature, presenting the phenotypic hallmarks of BS. However, skin lesions and upper airway infections were observed only in some of the patients. Overall, patients with pathogenic BLM variants had a more severe BS phenotype compared to patients carrying the pathogenic variants in RMI1, especially in terms of immunodeficiency, which should be considered as one of the most important phenotypic characteristics of BS.
Abnormal placental cord insertion, hypertensive disorders of pregnancy and birth length may be involved in development of hypospadias in male fetuses

Hypospadias is a congenital disease characterized by morphological abnormalities of the penis, including abnormal urethral opening and penile flexion, which cause urination disorders and/or sexual intercourse difficulty. Various factors have been suggested to cause this anomaly, but evidence concerning risk factors causing this anomaly is insufficient. We evaluated the etiology of hypospadias in Japan using the Common Database of the National Hospitals' Neonatal study group.
BARRIERS TO INDUSTRIALIZED BUILDING SYSTEM (IBS): THE CASE OF MALAYSIA

The benefits of Industrialised Building System (IBS) as a modern method of construction are numerous and far reaching. Reduced construction time, better site management, reduced wastage are but a few of these benefits, that will ultimately produce better products for the customers. The Malaysian government has spared no effort to bring IBS to the drawing tables of all professionals involved in the built environment. The IBS Roadmap 2003 -2010 has been endorsed by the Cabinet of Ministers to be the blueprint document for the industrialisation of the Malaysian construction sector. Nevertheless, towards the last two years of the roadmap period, the effort to promote the usage of IBS as an alternative to conventional and labour intensive construction method has yet to make headway. Although members of the industry are open to the idea, a major portion of the industry stakeholders are indifferent, perhaps due to resistance towards change, insufficient information and lack of technology transfer methods to support feasibility of change to IBS. Thus, the paper reviews literatures conserving barriers on implementing IBS in Malaysia which requires attention from different parties. Then, the paper validates the barriers identified in the literatures through pilot study with the practitioners from the industry. The barriers highlighted from both literatures and pilot study are negative perception, readiness issues, cost and equipment, poor planning and regulations, poor knowledge and awareness issues. Finally, the paper proposes recommendations to improve IBS take up in Malaysia. IBS in Malaysia may requires serious marketing and re-branding efforts, restructuring in R&D focus, reorganizing training and awareness program and proper incentive for IT adoption.
Hyperglucagonemia in Pediatric Adiposity Associates With Cardiometabolic Risk Factors but Not Hyperglycemia

Abstract Context In adults, hyperglucagonemia is associated with type 2 diabetes, impaired glucose tolerance, and obesity. The role of glucagon in pediatric overweight/obesity remains unclear. Objective We examined whether fasting concentrations of glucagon are elevated in youth with overweight/obesity and whether this associates with cardiometabolic risk profiles. Methods Analyses were based on the cross-sectional HOLBAEK study, including children and adolescents 6 to 19 years of age, with overweight/obesity from an obesity clinic group (n = 2154) and with normal weight from a population-based group (n = 1858). Fasting concentrations of plasma glucagon and cardiometabolic risk outcomes were assessed, and multiple linear and logistic regressions models were performed. Results The obesity clinic group had higher glucagon concentrations than the population-based group (P < 0.001). Glucagon positively associated with body mass index (BMI) standard deviation score (SDS), waist, body fat %, liver fat %, alanine transaminase (ALT), high-sensitivity C-reactive protein, homeostasis model assessment of insulin resistance, insulin, C-peptide, LDL-C, triglycerides, SDS of diastolic and systolic blood pressure, and was inversely associated with fasting glucose. The inverse relationship between glucagon and glucose was attenuated in individuals with high BMI SDS and high fasting insulin. Glucagon was associated with a higher prevalence of insulin resistance, increased ALT, dyslipidemia, and hypertension, but not with hyperglycemia. Glucagon was positively associated with fasting total glucagon-like peptide-1. Conclusion Compared with normal weight peers, children and adolescents with overweight/obesity had elevated concentrations of fasting glucagon, which corresponded to worsened cardiometabolic risk outcomes, except for hyperglycemia. This suggests hyperglucagonemia in youth may precede impairments in glucose regulation.
Susceptibility of Some Walnut Cultivars to Gnomonia leptostyla and Xanthomonas arboricola pv. juglandis in Bulgaria

The aim of the present research was to study and compare the susceptibility of 13 walnut cultivars – 5 Bulgarian (B), 3 French (F), 2 Hun­garian (H), and 3 American (A) – to Gnomonia leptostyla (Fr.) and Xanthomonas arboricola pv. juglandis (Pierce) Dye, the pathogens causing leaf spot and walnut blight. The study was conducted under natu­ral environmental conditions in a 5-8-year-old walnut collection orchard of the Fruit Growing Institute – Plovdiv, during the period 2006-2010. The evaluation of the attack produced by these pathogens was carried out on different organs leaves and nuts in two periods of the year (June and October). All the studied cultivars were distributed in 6 different levels of susceptibility to a given pathogen based on the degree of attack. The article presents data on the sensitivity of the studied walnut cultivars to the attack to G. leptostyla (Fr.) and X. arboricola pv. juglandis (Pierce) Dye and discusses the results obtained.
Genome-wide association studies dissect the genetic architecture of seed shape and size in common bean

Abstract Seed weight and size are important yield components. Thus, selecting for large seeds has been a key objective in crop domestication and breeding. In common bean, seed shape is also important since it influences industrial processing and plays a vital role in determining the choices of consumers and farmers. In this study, we performed genome-wide association studies on a core collection of common bean accessions to dissect the genetic architecture and identify genomic regions associated with seed morphological traits related to weight, size, and shape. Phenotypic data were collected by high-throughput image-based approaches, and utilized to test associations with 10,362 single-nucleotide polymorphism markers using multilocus mixed models. We searched within genome-associated regions for candidate genes putatively involved in seed phenotypic variation. The collection exhibited high variability for the entire set of seed traits, and the Andean gene pool was found to produce larger, heavier seeds than the Mesoamerican gene pool. Strong pairwise correlations were verified for most seed traits. Genome-wide association studies identified marker–trait associations accounting for a considerable amount of phenotypic variation in length, width, projected area, perimeter, and circularity in 4 distinct genomic regions. Promising candidate genes were identified, e.g. those encoding an AT-hook motif nuclear-localized protein 8, type 2C protein phosphatases, and a protein Mei2-like 4 isoform, known to be associated with seed size and weight regulation. Moreover, the genes that were pinpointed are also good candidates for functional analysis to validate their influence on seed shape and size in common bean and other related crops.
Effects of hyaluronic acid injections on pain and functioning in patients affected by tendinopathies: A narrative review.

BACKGROUND Tendinopathies are overuse tendon injuries showing load-dependant pain, stiffness, weakness of movement in the affected area, and impairment in the movements. The scientific interest on the role of Hyaluronic Acid (HA) for the management of tendinopathies has been increased due to its anti-inflammatory and lubricative properties. OBJECTIVE To collect evidence regarding the effectiveness and safety of HA injections in reducing pain in patients affected by tendinopathies. METHODS A scientific literature search was conducted using the PubMed, Medline and PEDro electronic databases. The databases were searched since their inception until July 2021. The search was limited to English language articles. Different combinations of the terms and MeSH terms "tendinopathy", "tendinosis", "tendinitis", "hyaluronic acid", "hyaluronate", "infiltration", "hyaluronic injections", "viscosupplementation" connected with various boolean operators were used for other electronic databases. RESULTS One hundred and one records were identified from the selected databases plus three additional papers identified by the authors through other sources. After removing duplicated papers and title/abstract screening, 19 studies were included in our review (eight papers on shoulder, three on elbow, four on hand, one on knee, and three on ankle). CONCLUSION The results showed that none of the studies report severe adverse effects and most of them support the use of HA injections in tendinopathies, with a special attention to pain reduction and functional assessment. Further studies are warranted to better investigate effects and methods of administration of HA in tendinopathies.
A critical analysis of research methods and biological experimental models to study pulp regeneration

Abstract With advances in knowledge and treatment options, pulp regeneration is now a clear objective in clinical dental practice. For this purpose, many methodologies have been developed in attempts to address the putative questions raised both in research and in clinical practice. In the first part of this review, laboratory‐based methods will be presented, analysing the advantages, disadvantages, and benefits of cell culture methodologies and ectopic/semiorthotopic animal studies. This will also demonstrate the need for alignment between two‐dimensional and three‐dimensional laboratory techniques to accomplish the range of objectives in terms of cell responses and tissue differentiation. The second part will cover observations relating to orthotopic animal studies, describing the current models used for this purpose and how they contribute to the translation of regenerative techniques to the clinic.
Suicidal and nonsuicidal self-harming thoughts and behaviors in clinically referred children and adolescents with gender dysphoria

Background Young transgender or gender nonconforming (TGNC) children and adolescents or those with a gender dysphoria (GD) diagnosis show an increased risk of suicidal and nonsuicidal self-harming thoughts and behaviors (STBs). Data from German-speaking regions on this topic and studies comparing self- and parent-reported STBs have been scarce. The study aims were therefore to examine: (a) frequencies of self- and parent-reported STBs in a German clinical sample of children and adolescents with GD and (b) differences between age and gender groups, as well as between self- and parent-reports. Methods Two items from the Child Behavior Checklist (CBCL) and the Youth Self-Report (YSR) were used to assess self- and parent-reported STBs in a sample of 343 adolescents and 49 children who presented to the Hamburg Gender Identity Service (Hamburg GIS) between 2013 and 2019. Seventy-eight percent of the analysis sample was assigned female at birth (AFAB), and 22% were assigned male at birth (AMAB). Results Parents reported STBs on the CBCL for 16% and 6% of the cases in childhood and for 20% and 29% of the adolescent cases, respectively. STBs were self-reported by 38% and 45% of the adolescents who could report on the YSR. STBs were higher among adolescents than among children and in self-reports (YSR) compared to parent reports (CBCL). AFAB adolescents reported higher degrees of STBs than AMAB adolescents. Conclusions Children and adolescents with GD are a high-risk population for STBs, especially pubescent and AFAB individuals. Mental health professionals should screen early for STBs to prevent possible suicidal ideation-to-action transitions.