Miracle-dz/UniFAR-FTUs huggingface dataset:
The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy.

Mitochondrial long chain fatty acid beta-oxidation provides the major source of energy in the heart. Deficiencies of human beta-oxidation enzymes produce sudden, unexplained death in childhood, acute hepatic encephalopathy, skeletal myopathy, or cardiomyopathy. Long chain 3-hydroxyacyl-CoA dehydrogenase [LCHAD; long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase, EC 1.1. 1.211] catalyzes the third step in beta-oxidation, and this activity is present on the C-terminal portion of the alpha subunit of mitochondrial trifunctional protein. We used single-stranded conformation variance analysis of the exons of the human LCHAD (alpha subunit) gene to determine the molecular basis of LCHAD deficiency in three families with children presenting with sudden unexplained death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome). In all families, the mothers had acute fatty liver and associated sever complications during pregnancies with the affected infants. The analysis in two affected children revealed a G to C mutation at position 1528 (G1528C) of the alpha subunit of the trifunctional protein on both alleles. This is in the LCHAD domain and substitutes glutamine for glutamic acid at position 474 of mature alpha subunit. The third child had this G1528C mutation on one allele and a different mutation (C1132T) creating a premature termination codon (residue 342) on the second allele. Our results demonstrate that mutations in the LCHAD domain of the trifunctional protein alpha subunit in affected offspring are associated with maternal acute fatty liver of pregnancy. This is the initial delineation of the molecular basis of isolated LCHAD deficiency.
A novel method of isolation, preservation, and expansion of human corneal endothelial cells.

PURPOSE To explore new strategies for effective isolation, preservation, and expansion of human corneal endothelial cells (HCECs). METHODS Human corneal Descemet's membrane and corneal endothelial cells were digested with collagenase A or Dispase II in supplemented hormonal epithelial medium (SHEM) for 1.5 to 16 hours. HCEC aggregates derived from collagenase A digestion were preserved in serum-free medium with low or high calcium for up to 3 weeks. Cryosections of HCEC aggregates were subjected to immunostaining with ZO-1, connexin 43, type IV collagen, laminin-5, and perlecan, and apoptosis was determined by TUNEL or cell-viability assay. For expansion, HCEC aggregates were seeded directly or after brief treatment with trypsin/EDTA in SHEM, with or without additional bovine pituitary extract (BPE), nerve growth factor (NGF), or basic fibroblast growth factor (bFGF). The resultant HCECs were immunostained with ZO-1, connexin 43, and Ki67. RESULTS Digestion with collagenase A, but not Dispase, of the stripped Descemet's membrane generated HCEC aggregates, which preserved cell-cell junctions and basement membrane components. High cell viability of HCEC aggregates was preservable in a serum-free, high-calcium, but not low-calcium, medium for at least 3 weeks. Brief treatment of HCEC aggregates with trypsin/EDTA resulted in a higher proliferation rate than without, when cultured in SHEM, and the resultant confluent monolayer of hexagonal cells retained cell-cell junctions. However, additional BPE, NGF, or bFGF did not increase cell proliferation, whereas additional BPE or bFGF disrupted cell-cell junctions. CONCLUSIONS Collagenase A digestion successfully harvested aggregates with viable HCECs that were preservable for at least 3 weeks in a serum-free, high-calcium medium and, with brief trypsin/EDTA treatment, expanded in the SHEM into a monolayer with hexagonal cells that exhibited characteristic cell junctions.
The role of student counselling in school leadership : case study in Finnish and Russian schools

Mironova, Sima. 2016. The Role of Student Counselling in School Leadership. Case study in Finnish and Russian schools. Master's Thesis in Education. University of Jyväskylä. Institute of Educational Leadership. The aim of my master's thesis research is to understand student counselling phenomenon and its role in Russian and Finnish school leadership. This research study shows differences of organising student counselling in schools itself and its role in a school leadership. Lifelong learning as a part of student counselling provides deep understanding of a counselling process and its importance of student's development. Qualitative research method is chosen as a research method and semistructured interview as a data collection method. There were a need of conducting six interviews in one Finnish school and five interviews in one Russian school. Thematic coding and network analysis were used to answer the four research questions. The results of case studies show that the student counselling is a part of school leadership in Finnish school, there is no place for it in Russian school leadership. Lifelong learning in Finnish perspective has accent on student's encouragement to be independent thinkers and continuous learners. Russian school takes a passive role in student’s readiness for lifelong learning. The further recommendation for study is to investigate lifelong-learning concept and to conduct research which will show the influence of lifelonglearning policies in educational systems on student's performance at school.
Loss of fornix white matter volume as a predictor of cognitive impairment in cognitively normal elderly individuals.

IMPORTANCE Magnetic resonance imaging markers of incipient cognitive decline among healthy elderly individuals have become important for both clarifying the biological underpinnings of dementia and clinically identifying healthy individuals at high risk of cognitive decline. Even though the role of hippocampal atrophy is well known in the later stages of decline, the ability of fornix-hippocampal markers to predict the earliest clinical deterioration is less clear. OBJECTIVES To examine the involvement of the hippocampus-fornix circuit in the very earliest stages of cognitive impairment and to determine whether the volumes of fornix white matter and hippocampal gray matter would be useful markers for understanding the onset of dementia and for clinical intervention. DESIGN A longitudinal cohort of cognitively normal elderly participants received clinical evaluations with T1-weighted magnetic resonance imaging and diffusivity scans during repeated visits over an average of 4 years. Regression and Cox proportional hazards models were used to analyze the relationships between fornix and hippocampal measures and their predictive power for incidence and time of conversion from normal to impaired cognition. SETTING A cohort of community-recruited elderly individuals at the Alzheimer Disease Center of the University of California, Davis. PARTICIPANTS A total of 102 cognitively normal elderly participants, with an average age of 73 years, recruited through community outreach using methods designed to enhance ethnic diversity. MAIN OUTCOMES AND MEASURES Our preliminary hypothesis was that fornix white matter volume should be a significant predictor of cognitive decline among normal elderly individuals and that fornix measures would be associated with gray matter changes in the hippocampus. RESULTS Fornix body volume and axial diffusivity were highly significant predictors (P = .02 and .005, respectively) of cognitive decline from normal cognition. Hippocampal volume was not significant as a predictor of decline but was significantly associated with fornix volume and diffusivity (P = .004). CONCLUSIONS AND RELEVANCE This could be among the first studies establishing fornix degeneration as a predictor of incipient cognitive decline among healthy elderly individuals. Predictive fornix volume reductions might be explained at least in part by clinically silent hippocampus degeneration. The importance of this finding is that white matter tract measures may become promising candidate biomarkers for identifying incipient cognitive decline in a clinical setting, possibly more so than traditional gray matter measures.
Information Sharing in the Supply Chains of Products With Seasonal Demand

This study considers a two-echelon supply chain with one supplier and one retailer for products with seasonal demand. The effects that information sharing has on coordination and benefits of the supply chain are investigated. In order to better forecast the seasonal demand, the supplier initiates the information sharing process by offering incentives to the retailer which are proportional to the degree of information sharing. Since the variance in the supplier's inventory would marginally decrease as the degree of information sharing increases, the benefits gained by the supplier due to information sharing are thus a convex function. This can be used to obtain the optimal degree of information sharing with the aim of maximizing profits, which is a tradeoff between the benefits gained and costs incurred by information sharing. In this study, the seasonal demand is described by a SARMA time series model. Bayesian analysis is employed to investigate the value of information and determine the optimal degree of information sharing. Constructive properties are derived to provide managerial insight for effective decision making. The results of sensitivity analyses show that the correlations of demand for successive periods and estimation errors would both have great effects on the benefits gained by information sharing.
Analysis of CAPZA3 localization reveals temporally discrete events during the acrosome reaction

In mammals, the starting point of development is the fusion between sperm and egg. It is well established that sperm fuse with the egg through the equatorial/post‐acrosomal region. Apart from this observation and the requirement of two proteins (CD9 in the egg and IZUMO1 in the sperm) very little is known about this fundamental process. Actin polymerization correlates with sperm capacitation in different mammalian species and it has been proposed that F‐actin breakdown is needed during the acrosome reaction. Recently, we have presented evidence that actin polymerization inhibitors block the movement of IZUMO1 that accompany the acrosome reaction. These results suggest that actin dynamics play a role in the observed changes in IZUMO1 localization. This finding is significant because IZUMO1 localization in acrosome‐intact sperm is not compatible with the known location of the initiation of the fusion between the sperm and the egg. To further understand the actin‐mediated changes in protein localization during the acrosome reaction, the distribution of the sperm‐specific plus‐end actin capping protein CAPZA3 was analyzed. Like IZUMO1, CAPZA3 shows a dynamic pattern of localization; however, these movements follow a different temporal pattern than the changes observed with IZUMO1. In addition, the actin polymerization inhibitor latrunculin A was unable to alter CAPZA3 movement. J. Cell. Physiol. 224: 575–580, 2010. © 2010 Wiley‐Liss, Inc.
Evaluating Individual Training Adaptation With Smartphone-Derived Heart Rate Variability in a Collegiate Female Soccer Team

Abstract Flatt, AA and Esco, MR. Evaluating individual training adaptation with Smartphone-derived heart rate variability in a collegiate female soccer team. J Strength Cond Res 30(2): 378–385, 2016—Monitoring individual responses throughout training may provide insight to coaches regarding how athletes are coping to the current program. It is unclear if the evolution of heart rate variability (HRV) throughout training in team-sport athletes can be useful in providing early indications of individual adaptation. This study evaluated relationships between changes in resting cardiac autonomic markers derived from a novel smartphone device within the first 3 weeks of a 5-week conditioning program and the eventual change in intermittent running performance at week 5 among 12 collegiate female soccer players. Change variables from weeks 1 to 3 of the weekly mean and weekly coefficient of variation for resting heart rate ([INCREMENT]RHRmean and [INCREMENT]RHRcv, respectively) and log-transformed root mean square of successive R-R intervals multiplied by 20 ([INCREMENT]Ln rMSSDmean and [INCREMENT]Ln rMSSDcv, respectively) were compared with changes in Yo-Yo Intermittent Recovery Test Level 1 performance ([INCREMENT]Yo-Yo). A very large and significant correlation was found between [INCREMENT]Yo-Yo and [INCREMENT]Ln rMSSDcv (r = −0.74; p = <0.01) and a large nonsignificant correlation was found with [INCREMENT]Ln rMSSDmean (r = 0.50; p = 0.096). This study suggests that a decrease in Ln rMSSDcv within the first 3 weeks of training is a favorable response, indicative of positive adaptation. Collecting daily HRV data with a smartphone application using ultrashort HRV measures seems useful for athlete monitoring.
Implementing Prover-Side Proximity Verification for Strengthening Transparent Authentication

Transparent authentication schemes based on proximity verification over a wireless channel are susceptible to relay attacks. In recent literature several countermeasures have been proposed. However these come with drawbacks in terms of usability and deployability. In this demo, we show a prototype implementation of STASH, a scheme for securing transparent authentication schemes using prover-side proximity verification, presented at SECON 2017.
Hop count discovery protocol for gradient based routing in wireless sensor networks

Wireless sensor networks are a challenging research field for the telecommunication engineer. The limited resources of the individual sensor nodes impose a redesign of the traditional wireless network protocols. These new designs aim primarily at reducing energy consumption and increasing scalability. Several gradient based routing protocols have been proposed in literature. They construct a cost field towards the sink to be able to forward data along the steepest gradient. A naive way to set up the cost field is flooding. In this paper we present an alternative approach based on received signal strength measurements and timers. The field is constructed in "consecutive waves" emanating from the initializing sink node. The considered cost is a combined metric which takes into account the minimum hop distance to the sink. A simulation study, using the OMNET++ network simulator environment, shows the feasibility, discusses the strengths and possible weaknesses of the proposed scheme and illustrates its energy efficient operation.
Hypothyroidism induces Fos-like immunoreactivity in ventral medullary neurons that synthesize TRH

Yuan, Pu-Qing, and Hong Yang. Hypothyroidism induces Fos-like immunoreactivity in ventral medullary neurons that synthesize TRH. Am. J. Physiol. 277 (Endocrinol. Metab. 40): E927–E936, 1999. —Altered thyroid statuses are associated with autonomic disorders. Thyrotropin-releasing hormone (TRH) in medullary nuclei regulates vagal efferent activity. Induction of Fos-like immunoreactivity (IR) in medullary TRH-synthesizing neurons was investigated in 24-h fasted rats with different thyroid statuses. Hypoand hyperthyroidism were induced by 6-N-propyl-2-thiouracil (PTU) in drinking water and a daily intraperitoneal injection of thyroxine (T4; 10 μg·100 g21 ·day21), respectively, for 1–4 wk. The numbers of Fos-like IR positive neurons in the raphe pallidus, raphe obscurus, and parapyramidal regions, which were low in euthyroid rats (0–2/section), increased remarkably as the hypothyroidism progressed and were negatively correlated with serum T4 levels. At the 4th wk, Fos-like IR positive neurons were 10to 70-fold higher compared with euthyroid controls. Simultaneous T4 replacement (2 μg·100 g21 ·day21) prevented the increases of Fos-like IR in PTU-treated rats. Hyperthyroidism did not change the number of Fos-like IR neurons in the raphe nuclei but reduced it in the parapyramidal regions. Double immunostaining revealed that most of the Fos-like IR induced by hypothyroidism was located in the prepro-TRH IR positive neurons. The selective and sustained induction of Fos-like IR in TRH-synthesizing neurons in ventral medullary nuclei by hypothyroidism indicates that these neurons play a role in the autonomic disorders observed in altered thyroid statuses.
[Chronic anterior laxity of the knee treated with free patellar graft and extra-articular lateral plasty: 10-year follow-up of 148 cases].

PURPOSE OF THE STUDY Assess long-term subjective, functional and radiographic outcome after free patellar graft and extra-articular lateral plasty for chronic anterior laxity of the knee. PATIENTS AND METHODS 148 cases of chronic anterior laxity of the knee treated by free patellar graft and extra-articular lateral Lemaire plasy were reviewed after a mean follow-up of 11.5 years (range 10-15 years). A complete work-up was performed in all cases at 4 years postsurgery to assess objective, sport, function and radiographic outcome (objective laxity and osteoarthritic status). RESULTS Subjectively, 65 p. 100 of the patients were very satisfied and 24 p. 100 were satisfied. According to the IKDC classification, functional outcome was in class A in 22 p. 100 and in class B in 49 p. 100. All failures (14 p. 100) except one occurred during the first year. When the meniscus was healthy or repaired, the failure rate was only 4 p. 100. Two principal factors favoring failure were severe laxity (meniscectomy) and poor femoral position. Residual laxity measured on the lateral view in the one-leg weight bearing position was 3.3 mm in the overall series, 2.4 mm for cases with isolated anterior laxity, and 4 mm for chronic laxities. Residual laxity was higher if the medial meniscus was totally or partially removed. There was no change between the 4th and 11th year of follow-up. A secondary meniscectomy was performed in 5 p. 100 of the cases despite renewed sports activity in 80 p. 100 of the cases. Osteoarthritic degeneration was the most important factor for less favorable outcome: 42 p. 100 of the cases developed preosteoarthitis or osteoarthritis. Joint degeneration occurred almost exclusively in patients who had undergone medial meniscectomy. Only 2 p. 100 of the patients with a healthy or repaired meniscus developed osteoarthritis. When the anterior laxity was the only anomaly, the functional result was very excellent, with renewed sports activity. Even in cases with persistent residual laxity, there were almost no failures or secondary meniscal lesions if the femoral position was correct. DISCUSSION For chronic laxity, free patellar graft alone cannot avoid a high rate of failure and/or joint degeneration, particularly favored by an incorrect femoral position. Improved results can only be achieved by preserving the meniscus and possibly associating a lateral or medial plasty whose effect remains to be evaluated.
Fluctuating asymmetry, sexual selection and canine teeth in primates

Fluctuating asymmetry arises as small deviations from symmetry which can be expressed on either side of the body. Increases in fluctuating asymmetry can suggest genomic stress such as results from directional selection. It has been argued that epigamic structures and weapons should show high levels of fluctuating asymmetry because sexual selection is essentially directional in nature. We tested this prediction by examining the expression of fluctuating asymmetry in the upper canines of 21 species of Old World primates. We found, for males but not for females, that asymmetry was correlated with measures of sexual selection including canine dimorphism, canine size, mass dimorphism, and intra-male competition. However, there was no significant correlation with diet type and body mass, which are only weakly associated with sexual selection. Phylogenetic inertia did not account for the association between fluctuating asymmetry and sexual selection. We also found that species with high values of canine dimorphism and intra-male competition tended to have a negative correlation between asymmetry and mean canine height, and this latter effect was present in both males and females. The implications of these findings for sexual selection theory are discussed.
Effects of erythropoietin on reducing brain damage and improving functional outcome after traumatic brain injury in mice.

OBJECT This study was designed to investigate the beneficial effects of recombinant human erythropoietin (rhEPO) treatment of traumatic brain injury (TBI) in mice. METHODS Adult male C57BL/6 mice were divided into 3 groups: 1) the saline group (TBI and saline [13 mice]); 2) EPO group (TBI and rhEPO [12]); and 3) sham group (sham and rhEPO [8]). Traumatic brain injury was induced by controlled cortical impact. Bromodeoxyuridine (100 mg/kg) was injected daily for 10 days, starting 1 day after injury, for labeling proliferating cells. Recombinant human erythropoietin was administered intraperitoneally at 6 hours and at 3 and 7 days post-TBI (5000 U/kg body weight, total dosage 15,000 U/kg). Neurological function was assessed using the Morris water maze and footfault tests. Animals were killed 35 days after injury, and brain sections were stained for immunohistochemical evaluation. RESULTS Traumatic brain injury caused tissue loss in the cortex and cell loss in the dentate gyrus (DG) as well as impairment of sensorimotor function (footfault testing) and spatial learning (Morris water maze). Traumatic brain injury alone stimulated cell proliferation and angiogenesis. Compared with saline treatment, rhEPO significantly reduced lesion volume in the cortex and cell loss in the DG after TBI and substantially improved recovery of sensorimotor function and spatial learning performance. It enhanced neurogenesis in the injured cortex and the DG. CONCLUSIONS Recombinant human erythropoietin initiated 6 hours post-TBI provided neuroprotection by decreasing lesion volume and cell loss as well as neurorestoration by enhancing neurogenesis, subsequently improving sensorimotor and spatial learning function. It is a promising neuroprotective and neurorestorative agent for TBI and warrants further investigation.
Cost analysis of initial deployment strategies for virtualized mobile core network functions

A virtualized network infrastructure is composed of multiple virtualized network functions (VNF) interconnected by well defined interfaces, and thus forming a VNF-graph. The initial deployment of such a VNF-graph inside a data center (DC) is a complex task with multidimensional aspects compared to deploying a single VNF that may represent a single network function. The problem space becomes more complex when each VNF is further decomposed into multiple VNF components (VNFC), where each VNFC embodies a subset of network functions attributed to the respective VNF. The challenge is to ensure that the deployment strategy meets the intra-functional constraints between the multiple VNFCs constituting the VNF-graph while ensuring service, performance and operational integrity, and also ensures optimal utilization of the underlying resources of the DC infrastructure (DCI). In this article we analyze the cost incurred by two “constraint-based heuristically applied” initial VNF/VNFC deployment strategies with reference to a virtualized mobile network infrastructure providing EPCaaS (evolved packet core as a service) while taking into consideration functional and administrative constraints. The cost of deployment is measured in terms of the utilization of DC infrastructure resources such as compute and networking. We also present the discussion in view of the ETSI NFV MANO framework, undergoing standardization, that is responsible for management and orchestration of NFV systems including VNF deployment.