Miracle-dz/UniFAR-FTUs huggingface dataset:
The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

Unusual Aggregates Several recent papers have revealed the unexpected genetic and pathological overlap between frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The most common genetic cause is the GGGGCC hexanucleotide repeat expansion upstream of the C9orf72 coding region affecting about 10% of all patients. It is currently unknown how repeat expansion might lead to neurodegeneration. C9orf72 patients show two distinct types of ubiquitinated inclusions in the central nervous system, one of which was identified as phosphorylated TDP-43 protein. However, all inclusions in the cerebellum and most inclusions in the hippocampus and neocortex lack TDP-43, and the actual disease protein is unknown. Mori et al. (p. 1335, published online 7 February; see the Perspective by Taylor) discovered that most of these characteristic inclusions contain poly-(Gly-Ala) and, to a lesser extent, poly-(Gly-Pro) and poly-(Gly-Arg) dipeptide-repeat proteins that are generated by non-ATG–initiated translation from the expanded GGGGCC repeats in three reading frames. The findings yield mechanistic insight into the pathogenesis of FTLD/ALS with C9orf72 repeat expansions and directly link this common mutation to the characteristic pathology. A new class of proteins links a common genetic mutation to the predominant pathology in certain neurodegenerative diseases. [Also see Perspective by Taylor] Expansion of a GGGGCC hexanucleotide repeat upstream of the C9orf72 coding region is the most common cause of familial frontotemporal lobar degeneration and amyotrophic lateral sclerosis (FTLD/ALS), but the pathomechanisms involved are unknown. As in other FTLD/ALS variants, characteristic intracellular inclusions of misfolded proteins define C9orf72 pathology, but the core proteins of the majority of inclusions are still unknown. Here, we found that most of these characteristic inclusions contain poly-(Gly-Ala) and, to a lesser extent, poly-(Gly-Pro) and poly-(Gly-Arg) dipeptide-repeat proteins presumably generated by non-ATG–initiated translation from the expanded GGGGCC repeat in three reading frames. These findings directly link the FTLD/ALS-associated genetic mutation to the predominant pathology in patients with C9orf72 hexanucleotide expansion.
Factors affecting the accuracy of ultrasonographical fetal weight estimation in twin pregnancies

Objective. To determine the factors affecting the accuracy of ultrasonographic weight estimation in twins. Methods. 152 sets of twins delivered vaginally, were included. Effects of fetal weights, inter-twin weight discordance, chorionicity, early rupture of membranes, intrauterine growth restriction, and presentations of twins on errors of estimated fetal weights were evaluated. The primary measures of estimated fetal weight accuracy compared were mean-percentage-error and the standart deviation (SD) of percentage errors. Results. Mean percentage errors for the first fetus (8.13 ± 6.82) and the second fetus (8.07 ± 6.88) were similar (p = 0.64). Random errors of both fetuses were also similar (p = 0.78). If one of the fetuses had IUGR, the percentage error and also the random error of that fetus would increase significantly. Different presentations and fetal gender combinations were similar for both types of errors of fetal weight estimation. A weak negative lineer relationship was found between the weight of the first fetus and its percentage error (r = −0.27, p = 0.04). A similar relation was present between the weight and percentage error of the second fetus (r = −0.29, p = 0.03). Percentage errors and also random errors of both fetuses were significantly higher if severe discordance was present between twins (p = 0.01 and p = 0.02, respectively). Conclusions. IUGR, fetal weights, and inter-twin discordence are the factors affecting the accuracy of weight estimation by ultrasonography.
Shocks on the Romanian Foreign Exchange Market Before and after the Global Crisis

This paper explores some changes induced on the Romanian foreign exchange market by the global crisis. We study these changes from the perspective of number and intensity of the shocks occurred before and after the global crisis. We found some significant differences, explainable not only by the direct effects of the crisis, but also by the intervention of the National Bank of Romania.
Informal Tradables and the Employment Growth of Indian Manufacturing

India's manufacturing growth from 1989 to 2010 displays two intriguing properties: 1) a substantial fraction of absolute and net employment growth is concentrated in informal tradable industries, and 2) much of this growth is connected to the development of one-person establishments. This paper investigates the causes and determinants of these growth patterns. The rapid urbanization of the informal sector plays the strongest role, while there is some evidence for subcontracting by the formal sector and a"push"entrepreneurship story. The paper also finds modest connections of this growth to rising female labor force participation. The connection between the presence of informal manufacturing and local productivity levels is strong, and varies across urban and rural areas in ways that bolster urbanization and subcontracting hypotheses.
Three essays on hedge fund fee contracts, managerial incentives and risk taking behaviors

DEDICATION To my loving, supportive and inspiring parents, Shihong Xie and Jie Zhan. v ACKNOWLEDGMENTS I would like to thank my doctoral advisor and dissertation committee chair, Dr. Bing Liang, for introducing me to the research field of hedge funds, and for his many years of thoughtful, patient guidance and support. " Be persistent. " is the first ever piece of advice he gave me, which I carried carefully and faithfully throughout my doctoral study, and will carry forward as faithfully in my academic career ahead. helpful comments and suggestions on all stages of this dissertation. Thanks are also due to other faculty members at Isenberg School of Management. All the professors mentioned above altogether have carved out an everlasting role model of a successful, knowledgeable, productive, creative, dedicated and student-centered finance scholar for me. I am in great debt to my parents. Their courage in facing and fighting against illness has been a great source of courage for me to meet the many challenges and difficulties during my academic pursuit these years. Under the principal-agent framework, the first essay studies and compares different compensation schemes commonly adopted by hedge fund and mutual fund managers. We find that the option-like performance fee structure prevalent among hedge funds is suboptimal to the symmetric performance fee structure. However, the use of high water mark (HWM) mitigates the suboptimality, though to a very limited extent. Both our theoretical models and simulation results show that HWM will induce more managerial efforts only when a fund is slightly under the water but it will unfavorably dampen incentives when a fund is too deep under the water and when the manager's skill is poor. Allowing managers to invest personal wealth in their own funds, however, helps align interests and provides positive managerial incentives. Existing literature has detected a " tournament behavior " among mutual fund managers that midyear underperformers tend to take relatively higher risk than peers in the second half-year. The second essay reexamines this issue and provides empirical evidence that such behavior does not exist among hedge fund managers, either at fund level or risk style level. Instead, hedge fund managers shift risk at midyear in response to the moneyness of their incentive contracts. vii Also, risk shifting decisions are more driven by underperformance than by outperformance. High Water Mark can strongly rein in excess risk-taking and therefore better aligns interests. Last, risk shifting …
Osteoporosis is not a specific complication of primary biliary cirrhosis (PBC)

Newton et al ( Gut 2001; 49 :282–7) described a retrospective study on bone mineral density (BMD) in a large cohort of patients with primary biliary cirrhosis (PBC). The authors concluded that osteoporosis is not a specific complication of PBC, but certain weaknesses in the study design do not support this conclusion. * The authors did not include age and sex matched controls from the general population, or control groups with different types of liver disease. * A proper methodological design comparing osteoporosis in PBC and in a normal population should calculate the standardised incidence ratio of osteoporosis for the two cohorts by comparing the observed incidence versus the expected incidence. The calculation should include 95% confidence intervals according to exact Poisson limits.1 * A logistic regression analysis including risk factors for osteoporosis (that is, age, menopausal status, smoking habits, alcohol consumption, etc) should have been performed. The major drawback however is the …
Candidate Tidal Dwarf Galaxies in the Compact Group CG J1720–67.8

This is the second part of a detailed study of the ultracompact group CG J1720-67.8. In the first part we focused attention on the three main galaxies of the group and identified a number of candidate tidal dwarf galaxies (TDGs). Here we concentrate on these candidate TDGs. Absolute photometry of these objects in BVRJHKs bands confirms their relatively blue colors, as we already expected from the inspection of optical and near-infrared color maps and from the presence of emission lines in the optical spectra. The physical conditions in such candidate TDGs are investigated through the application of photoionization models, while the optical colors are compared with grids of spectrophotometric evolutionary synthesis models from the literature. Although from our data self-gravitation cannot be proved for these objects, their general properties are consistent with those of other TDG candidates. In addition, we present the photometry of a few "knots" detected in the immediate surroundings of CG J1720-67.8 and consider the possibility that these objects might belong to a dwarf population associated with the compact group.
Lexicography in the Eighteenth-century Gran Chaco: the Old Zamuco Dictionary by Ignace Chomé

The Vocabulario de la lengua zamuca is the only extant dictionary of Old Zamuco, an extinct Zamucoan language spoken in the 18th century in the abandoned mission of San Ignacio de Samucos, located in the northern Chaco lowlands of South America. This document was until now inaccessible to scholars, but has now been thoroughly studied by the present author, and found to contain very rich data, which establish its fundamental importance for linguistic studies on Zamucoan and Chaco languages. The critical edition of the dictionary is currently under publication. The original author of the dictionary, the Jesuit Father Ignace Chome (1696-1768), reveals his brilliant linguistic intuition and remarkable skills in the collection and representation of linguistic data. One of the theoretical challenges he had to face was the threefold system of nominal suffixation, which is an absolute linguistic rarity. The present paper will show how the author dealt with the main word classes of Old Zamuco, that is verbs, nouns and adjectives. Chome structured the entries for these lexemes in such a way as to provide plenty of information on inflectional and derivational morphology. The data from the dictionary permit new and interesting insights on the grammar of Old Zamuco.
A Survey of Reproductive Management Strategies on US Commercial Dairy Farms

Reproductive performance on the dairy farm affects the dairy’s profit because it directly affects milk production, the availability of replacements, the amounts of voluntary and involuntary culling, breeding costs, and costs associated with veterinary care (Britt, 1985). Reproductive management programs selected for implementation differ across farms due to varying on-farm costs, such as labor costs, opportunity costs of management and labor, as well as facilities, farm goals and values, and management styles. The objective of this paper is to summarize survey data in order to aid in providing economic insight into why varying types of farms utilize different methods of reproductive management and differing reproductive technologies.
Correction: A New Type of Proton Coordination in an F1Fo-ATP Synthase Rotor Ring

The images for Figures S2 and S3 were incorrectly switched. The image that appears as Figure S2 should be Figure S3, and the image that appears as Figure S3 should be Figure S2. The figure legends appear in the correct order. Please view the correct Figure S2 here: Click here for additional data file. (1.5M, tif)
Alcohol, Frustration, and Direct Physical Aggression: A Methodological Point of View

An experiment tested the hypothesis that alcohol in moderate doses will interact with frustration and increase aggression measured within the Buss teacher-learner paradigm. The hypothesis was not possible to evaluate because subjects developed behavioral strategies to speed up learning by their partners. These strategies, which kept subjects from using high shocks, obscured potential effects of alcohol. The results were discussed in terms of methodology and a need for a modification of the standard learning “cover explanation. ”
Classical (adiponectin, leptin, resistin) and new (chemerin, vaspin, omentin) adipocytokines in patients with prediabetes

Abstract Background In the last decade, there has been an increased interest toward fat tissue as an endocrine organ that secretes many cytokines and bioactive mediators that play a role in insulin sensitivity, inflammation, coagulation and the pathogenesis of atherosclerosis. The aim of this study was to investigate classical (adiponectin, leptin, resistin) and new (chemerin, vaspin, omentin) adipocytokine levels in subjects with prediabetes [impaired fasting glucose (IFG) and/or impaired glucose tolerance (IGT)] and obese subjects with normoglycemia. Methods In this study, 80 patients with a mean age of 50.4 ± 10.6 years were recruited, divided into two groups with similar age and body mass index (BMI) – with obesity and normoglycemia (n = 41) and with obesity and prediabetes (n = 39). Results Serum adiponectin levels were significantly higher in subjects with normoglycemia compared to patients with prediabetes. Adiponectin has a good discriminating power to distinguish between patients with and without insulin resistance in our study population [area under the curve (AUC) = 0.728, p = 0.002]. Other adipocytokine levels were not significantly different between the two groups. The patients with metabolic syndrome (MetS) had significantly lower levels of leptin compared to those without MetS (33.03 ± 14.94 vs. 40.24 ± 12.23 ng/mL) and this difference persisted after adjustment for weight and BMI. Receiver operating characteristic (ROC) analysis showed that low serum leptin can predict the presence of MetS (p = 0.03), AUC = 0.645. Conclusion Serum adiponectin is statistically higher in patients with normoglycemia compared to those with prediabetes and has a predictive value for distinguishing between patients with and without insulin resistance in the studied population. Serum leptin has a good predictive value for distinguishing between patients with and without MetS in the studied population.
The Steady-State Level of the Nervous-System-Specific MicroRNA-124a Is Regulated by dFMR1 in Drosophila

Fragile X syndrome is the most common form of inherited mental retardation caused by loss of the fragile X mental retardation protein 1 (FMRP). The detailed molecular pathways underlying the pathogenesis of this disorder remain incompletely understood. Here, we show that miR-124a, a nervous-system-specific miRNA, is associated with the Drosophila homolog of FMRP (dFMR1) in vivo. Ectopic expression of wild-type but not mutant miR-124a precursors decreased dendritic branching of dendritic arborization sensory neurons, which was partially rescued by the loss of dFMR1 activity, suggesting that the biogenesis and/or function of miR-124a are partially dependent on dFMR1. Indeed, in contrast with the complete loss of mature miR-124a in Dicer-1 mutants, steady-state levels of endogenous or ectopically expressed mature miR-124a were partially reduced in dfmr1 mutants, whereas the level of pre-miR-124a increased. This effect could be explained in part by the reduced abundance of the Dicer-1-Ago1 complex in the absence of dFMR1. These findings suggest a modulatory role for dFMR1 to maintain proper levels of miRNAs during neuronal development.
Photoactivated toxicity in amphipods collected from polycyclic aromatic hydrocarbon–contaminated sites

The risk of photoactivated PAH toxicity in contaminated aquatic systems has not been well characterized. To document risk, amphipods (Gammarus spp.) were collected from two polycyclic aromatic hydrocarbon (PAH)‐contaminated sites in the lower St. Louis River and Duluth Harbor, USA (Hog Island and USX) as well as a reference site (Chipmunk Cove) and were exposed in two separate, replicate tests to controlled intensities of solar radiation for 3 d. Contaminated site organisms died significantly faster compared to control site organisms. In all tests, mortality was strongly related to ultraviolet‐A (UV‐A; 320–400 nm) dose. Ultraviolet‐B (280–320 nm) radiation did not increase mortality. To compare susceptibility among populations, regressions of arcsine‐transformed, proportionate mortality versus UV dose were completed for each, and the slopes were statistically compared. Response slopes for the two contaminated site populations were both significantly greater than the reference site population (p = 0.0001 for test 1; p = 0.0002 for test 2). These results indicate that organisms residing in PAH‐contaminated environments can accumulate PAH concentrations sufficient to be at risk for photoactivated toxicity. Although amphipods are not typically at risk of PAH‐photoactivated toxicity because they are largely protected from exposure to sunlight, they are representative surrogates for species that may be similarly protected at some life stages (and thus able to accumulate significant PAH tissue concentrations) but not at others.
Which Anhysteretic Magnetisation Curve?

The anhysteretic behaviour of nickel has been studied by two methods: the conventional field cycling method, and by thermal demagnetization followed by cooling in an applied field. The results show that the field cycling method does not achieve thermal equilibrium, the anhysteretic magnetizations differing by as much as 6% between the two methods. The results are discussed in terms of domain wall pinning.
A mobility-aware cross-layer congestion control, routing and scheduling design in ad-hoc wireless networks

In this paper, we consider the mobility effect with a jointly optimal design of cross-layer congestion control, routing and scheduling for ad-hoc networks. We first formulate the rate constraint and scheduling constraint. In this way, we use multi-commodity flow variables. Then formulate resource allocation in networks with fixed wireless channel and single-rate devices. Because of entrance of the effect mobility in optimal design, we formulate resource allocation as utility and cost function, together in a maximization problem with those constraints. By dual decomposition, decompose the resource allocation problem vertically into three sub-problems:, congestion control, routing and scheduling. These three sub-problems interact through congestion and link price.
Body Mass Index and Gonadotropin Hormones (LH & FSH) Associate With Clinical Symptoms Among Women With Polycystic Ovary Syndrome

To evaluate the relevance of body mass index (BMI), luteinizing hormone (LH), follicle-stimulating hormone (FSH) and LH/FSH ratio with clinical symptoms in polycystic ovary syndrome (PCOS) women. Methods: We reviewed the medical records of all women visited in the PCOS Clinic of Babol (Iran) from 2008 to 2012. A retrospective cross-sectional study was conducted on 175 PCOS women; aged 18–38 years diagnosed based on the Rotterdam criteria. Among the PCOs women, the prevalence of oligomenorrhea, acne, and hirsutism were found to be 92.0%, 31.4%, and 78.9%, respectively. Positive finding of polycystic ovaries was observed in 89.1% of PCOS women with by using sonography. A total of 69.2% overweight/obesity patients had polycystic ovary morphology on ultrasound image. Compared with non- overweight/obesity, the adjusted OR of PCOS women for sonographic view of polycystic ovaries was 4.33 (95% CI, 1.42-13.15, p=0.001), Nevertheless, the adjusted odds ratio (OR) showed no significant associations between LH, FSH, and LH/FSH ratio with clinical symptoms in these women. The findings of this study indicated that the overweight/obese women with PCOS are at an increased risk for sonographic view of polycystic ovaries. Therefore, it is suggested that successful weight loss is the most effective method of restoring ovulation, menstruation that should be used as major advice in obese PCOS patients.
In-Depth Temporal Transcriptome Profiling Reveals a Crucial Developmental Switch with Roles for RNA Processing and Organelle Metabolism That Are Essential for Germination in Arabidopsis1[W][OA]

Germination represents a rapid transition from dormancy to a high level of metabolic activity. In-depth transcriptomic profiling at 10 time points in Arabidopsis (Arabidopsis thaliana), including fresh seed, ripened seed, during stratification, germination, and postgermination per se, revealed specific temporal expression patterns that to our knowledge have not previously been identified. Over 10,000 transcripts were differentially expressed during cold stratification, with subequal numbers up-regulated as down-regulated, revealing an active period in preparing seeds for germination, where transcription and RNA degradation both play important roles in regulating the molecular sequence of events. A previously unidentified transient expression pattern was observed for a group of genes, whereby a significant rise in expression was observed at the end of stratification and significantly lower expression was observed 6 h later. These genes were further defined as germination specific, as they were most highly expressed at this time in germination, in comparison with all developmental tissues in the AtGenExpress data set. Functional analysis of these genes using genetic inactivation revealed that they displayed a significant enrichment for embryo-defective or -arrested phenotype. This group was enriched in genes encoding mitochondrial and nuclear RNA-processing proteins, including more than 45% of all pentatricopeptide domain-containing proteins expressed during germination. The presence of mitochondrial DNA replication factors and RNA-processing functions in this germination-specific subset represents the earliest events in organelle biogenesis, preceding any changes associated with energy metabolism. Green fluorescent protein analysis also confirmed organellar localization for 65 proteins, largely showing germination-specific expression. These results suggest that mitochondrial biogenesis involves a two-step process to produce energetically active organelles: an initial phase at the end of stratification involving mitochondrial DNA synthesis and RNA processing, and a later phase for building the better-known energetic functions. This also suggests that signals with a mitochondrial origin and retrograde signals may be crucial for successful germination.