Miracle-dz/UniFAR-FTUs huggingface dataset:
The ketogenic diet compensates for AGC1 deficiency and improves myelination

The brain aspartate‐glutamate carrier (AGC1) is specifically expressed in neurons, where it transports aspartate from the mitochondria to the cytosol, and plays a role in transfer of nicotinamide adenine dinucleotide (NADH)‐reducing equivalents into the mitochondria as a part of the malate‐aspartate shuttle. Deficient function of AGC1 underlies an inborn error of metabolism that presents with severe hypotonia, arrested psychomotor development, and seizures from a few months of age. In AGC1 deficiency, there is secondary hypomyelination due to lack of N‐acetylaspartate (NAA), which is normally generated by acetylation of aspartate in the neuron and required for fatty acid synthesis by the adjacent oligodendrocyte. Based on experiences from AGC2 deficiency, we predicted that reduced glycolysis should compensate for the metabolic defect and allow resumed myelination in AGC1 deficiency. Carbohydrate restriction was therefore initiated in a patient with AGC1 deficiency at 6 years of age by introducing a ketogenic diet. The response was dramatic, clinically as well as radiologically. Psychomotor development showed clear improvement, and magnetic resonance imaging (MRI) indicated resumed myelination. This is the first successful treatment of secondary hypomyelination reported. Because AGC1 is driven by the proton gradient generated by the neuronal mitochondrial respiratory chain, the results have potential relevance for secondary hypomyelination in general.
Decisional Procrastination in Academic Settings: The Role of Metacognitions and Learning Strategies

Nowadays, university students suffer from a broad range of problems, such as educational underachievement or the inability to control themselves, that lead to procrastination as a consequence. The present research aimed at analyzing the determinants of decisional procrastination among undergraduate students and at assessing a path model in which self regulated learning strategies mediated the relationship between metacognitive beliefs about procrastination and decisional procrastination. 273 students from Southern Italy filled out a questionnaire composed by: the socio-demographic section, the Metacognitive Beliefs About Procrastination Questionnaire, the procrastination subscale of the Melbourne Decision Making Questionnaire, and the Anxiety, the Time Management, and the Information Processing subscales of the Learning and Study Strategies Inventory. Results showed that the relationship between negative and positive metacognitive beliefs about procrastination and decisional procrastination was mediated only by time management and anxiety. Such findings underlined the crucial role played by learning strategies in predicting the tendency to delay decisional situations and in mediating the relationship between metacognitive beliefs about procrastination and decisional procrastination.
Developing the small business market for business development services in Chengdu

Purpose – The purpose of the paper is to assess the state of the business development services (BDS) market in Chengdu, with a view to identifying demand and/or supply side deficiencies, which public policy might help to address.Design/methodology/approach – The data are drawn from two surveys undertaken in Chengdu between November 2005 and January 2006.Findings – The research shows there are small firms that are active users of BDS, reflecting the emergence of non‐state owned enterprises in the city. The survey also shows evidence of unfulfilled latent demand, with a particular reference to sales and marketing advice and helping firms to access sources of finance. In addition, the survey of BDS providers shows that some are sensitive to the needs of small firms, although the latter are typically under‐represented in their client base.Practical implications – The findings suggest that the BDS market in Chengdu is underdeveloped from a small business perspective, which can be illustrated by pointing to bot.
Macro Spatial Modulation for Uplink mmWave Communication Systems

In this paper, we investigate the uplink of a millimeter-wave (mmWave) communication system, in which the small base stations (SBSs) are densely placed. We assume that a user terminal (UT) can be served by multiple SBSs and propose a macro spatial modulation (M-SM) scheme, in which the indexes of the SBSs are used to carry part of UTs information. The performance of the proposed M-SM scheme is investigated and an energy based detection method with reduced computational complexity is also provided. In addition, considering that certain SBSs may have similar array manifold vectors, an adaptive mode selection scheme, in which the UT switches between two different modes, is presented to further improve the system performance. Numerical results are provided to verify the performance of the proposed scheme. It is shown that compared with the conventional communication scheme in which the UT only communicates with the SBS with a stronger channel, the proposed M-SM scheme can achieve better performance.
Theoretical Computer

Tries and patricia tries are two popular data structures for storing strings. Let H n denote the height of the trie (the patricia trie, respectively) on a set of n strings. Under the uniform distribution model on the strings, it is well known that H n / log n → 2 for tries and H n / log n → 1 for patricia tries, when n approaches inﬁnity. Nevertheless, in the worst case, the height of a trie can be unbounded and the height of a patricia trie is in (cid:2)( n ) . To better understand the practical performance of both tries and patricia tries, we investigate these two classical data structures in a smoothed analysis model. Given a set S = { s 1 , s 2 , . , s n } of n binary strings, we perturb the set by adding an i.i. d. Bernoulli random noise to each bit of every string. We show that the resulting smoothed heights of the trie and the patricia trie are both in (cid:2)( log n ) .
Comprehensive analysis and evaluation to unsupervised binary hashing method in image similarity measurement

This study proposes an unsupervised binary hashing (UBH) method and provides a comprehensive analysis and evaluation to the UBH method in image similarity. Using the orthogonal locality preserving projection, the proposed UBH method performs the dimensionality reduction (DR). To reduce the quantisation error between low-dimensional vectors and binary hash codes generated in the DR, the proposed UBH method calculates the optimal parameters of rotation and offset. The authors use two test beds to evaluate the preservation of original feature space and the semantic consistency. Experimental results with two test beds show that the proposed UBH method is state of the art.
Managing industrial service co-design: identifying challenges from technology firms

This article investigates how the co-design of industrial services is managed by technology firms. Through multiple-case studies within high-technology semiconductor companies, the article finds that overlapped stages and collective action require considerations on a case-by-case basis as determined by business opportunities, industry standards, existing organisational practice and service level agreements. The major contribution of this article is a connection between recent research emphasising the importance of providing industrial services, and established literature on co-design. Key service operations management challenges are also identified and discussed in terms of the need for studies and frameworks to coordinate negotiations for enhanced service encounters, to critique industrial service co-design, and to integrated information during service design.
Human papillomavirus vaccination in survivors of childhood cancer

Effective vaccination is now available to prevent human papillomavirus (HPV), the most common sexually transmitted infection and the cause of cervical cancer, which is the second most common cancer among women worldwide. HPV vaccine uptake is particularly important for females surviving cancer, some of whom are at high risk for HPV complications because of the direct and indirect effects of cancer treatment. Thus, version 3.0 of the Children's Oncology Group's Long‐Term Follow‐Up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancer recommends HPV vaccination for all eligible females surviving childhood cancer. Because this vaccine was only approved by the US Food and Drug Administration in 2006, little is known regarding the complexity of vaccination uptake among those surviving cancer. The purpose of this article was to describe the HPV vaccine and its usefulness in the survivorship population, provide a rationale for describing cancer survivors as being at increased risk for HPV complications, identify factors associated with HPV vaccination, and discuss the utilization of these predictors in designing strategies to promote adherence to HPV vaccination recommendations within the survivorship context. Cancer 2009. © 2009 American Cancer Society.
New Method for Real Option Valuation Using Fuzzy Numbers

Real option analysis offers interesting insights on the value of assets and on the profitability of investments, which has made real options a growing field of academic research and practical application. Real option valuation is, however, often found to be difficult to understand and to implement due to the quite complex mathematics involved. Recent advances in modeling and analysis methods have made real option valuation easier to understand and to implement. This paper presents a new method for real option valuation using fuzzy numbers that is based on findings from earlier real option valuation methods and from fuzzy real option valuation. The method is intuitive to understand and far less complicated than any previous real option valuation model to date.
Can Gold Effectively Hedge Risks of Exchange Rate

This study tests whether gold can effectively hedge exchange rate risks. We take into account the asymmetric characteristic of exchange rate fluctuations and use the dynamic panel threshold model in order to select gold prices in major gold-related currencies in the world: the Australian dollar, the Canadian dollar, the euro, the Indian rupee, the Japanese yen, the South African rand, and the British pound. Using monthly data from January 1999 to January 2010, with lagged one-period exchange rate returns (US dollar depreciation rate) as the threshold variable, the estimation results suggest that there are two thresholds at -7.5% and -3.7%. These can be divided into regime 1 (exchange rate returns ≤ -7.5%), regime 2 (-7.5% > exchange rate returns ≤ -3.7%), and regime 3 (exchange rate returns > -3.7%). Regarding the effectiveness of gold hedging, regime 2 is higher than is regime 3. The risk hedging effect of regime 1 is not significant because it might be caused by the excessive devaluation of the US dollar in the short-term and the overshooting of the exchange rate adjustment, making gold unable to hedge the devaluation risks of the US dollar.
Perpendicular Cross-Spectra Fusion for Sound Source Localization With a Planar Microphone Array

Multiple sound source localization in reverberant environments stands as one of the most difficult challenges for many applications related to microphone array signal processing. In this paper, we describe perpendicular cross-spectra fusion (PCSF), a new direction-of-arrival (DOA) estimation algorithm, which utilizes an analytic formula for direction estimation in the time–frequency (TF) domain. Inherent to this technique is the presence of multiple direction estimation subsystems which operate in parallel, producing a multiplicity of candidate DOAs at each TF point. We define a metric of coherence, based on the property of divergence of the different DOA estimators, for assessing the reliability of different signal portions, so that only TF bins with a high quality of directional information are exploited for local DOA estimation. The resulting collection of local DOAs is provided as input to a recently proposed histogram processing approach, which is based on matching pursuit. Results based on simulation and real recordings illustrate the advantages of PCSF compared to other DOA estimation techniques subject to the same histogram-based processing, in the context of real-time multiple source localization and counting; improved performance in reverberant conditions and high tolerance to diffuse and common mode noise.
A plethora of presynaptic proteins associated with ATP‐storing organelles in cultured astrocytes

Cultured astrocytes can release a variety of messenger substances via receptor‐mediated mechanisms, implicating their potential for regulated exocytosis and the participation of proteins of the SNARE complex. Here we demonstrate the astrocytic expression and organellar association of a large variety of synaptic proteins (synaptobrevin II, synaptotagmin I, synaptophysin, rab3a, synapsin I, SNAP‐25, and syntaxin I) and also of the ubiquitous cellubrevin. As revealed by immunoblotting the expression of synaptic proteins was highest within the first few days after plating. Synaptophysin and SNAP‐25 showed the most significant decline with prolonged culture time. Rab3a and synaptobrevin II were retained at a high level and synaptotagmin I, synapsin I, and syntaxin I at a lower level until 20 DIV. The immunoreaction for cellubrevin was low at the beginning and increased with prolonged culture time. As revealed by light microscopical immunocytochemistry the proteins are expressed by GFAP‐positive astrocytes and associated with organelles of varying size. Immunoelectron microscopical analysis allocates synaptobrevin II and synaptophysin to the membranes of vesicular organelles. Double labeling experiments for pairs of synaptic proteins reveal that individual synaptic proteins can be entirely colocalized or partly reside on different organelles. Subcellular fractionation of astrocyte cultures by sucrose density gradient centrifugation after 2, 6, 13, and 20 DIV showed that the proteins sediment with ATP containing organelles of a broad density range. Our data suggest that messenger substances may be released from cultured astrocytes via receptor‐mediated, Ca2+‐dependent exocytosis. GLIA 26:233–244, 1999. © 1999 Wiley‐Liss, Inc.
How accurate is recall of key symptoms of depression? A comparison of recall and longitudinal reports

Background. Assessment of lifetime major depression is usually made from a single interview. Most previous studies have investigated reliability. Comparison of recall of key symptoms and longitudinal reports shows the accuracy of recall, not just reliability. Method. At age 25, 1003 members of the Christchurch Health and Development Study cohort were asked to recall key symptoms of depression (sadness, loss of interest) up to age 21. This recall was compared with longitudinal reports at ages 15, 16, 18 and 21 years. Diagnosis was by DSM-III-R and DSM-IV criteria. Results. Only 4% of those without previous reports recalled key symptoms. Of those with a diagnosis of depression up to age 21, 44% recalled a key symptom. Measures of severity of an episode (number of symptoms, impairment, duration, suicidality) and chronicity (years with a diagnosis, years with suicidal ideation) all strongly predicted recall. Current key symptoms increased recall, even after taking account of severity and chronicity. Being female and receiving treatment also predicted recall, although odds ratios were reduced to 1·6–1·7 when all other predictors were included. Comparison of risk factors for key symptoms showed similar results from longitudinal reports and recall. Sexual abuse, neuroticism, lack of parental attachment, gender, physical abuse and maternal depression were major risk factors in both sets of analyses. Conclusions. Forgetting of prior episodes of depression was common. Severity, chronicity, current depression, gender and treatment predicted recall. Lifetime prevalence based on recall will be markedly underestimated but the identification of major risk factors may be relatively little impaired.
Linkages among European and world stock markets

Causal relations and dynamic interactions among equity returns in ten countries for the period 1983-1994 are analysed. An innovation accounting approach based on a multivariate vector autoregressive (VAR) model is used to estimate the proportion of each market return's forecast error attributable to innovations in foreign market returns. Three major results appear. The variance decompositions indicate a strong degree of economic interaction among stock markets. The US stock market has a considerable influence on stock market performance in almost every country, while there is no substantial inter-continental influence from the European stock markets on the world's two largest equity markets in New York and Tokyo. Finally, the pattern of the impulse-response functions illustrates a rapid international transmission of stock market events, supporting the hypothesis of international stock market efficiency.
A population study using the human erythrocyte PIG‐A assay

Erythrocyte‐based PIG‐A assay is sensitive and reliable in detecting exposure to mutagenetic agents in animal studies, but there are few data from human populations. In this study, we employed a method for detecting CD59 phenotypic variants, resulting from mutation in the PIG‐A gene, in human red blood cells (RBCs), and determined the CD59‐deficient RBC (RBCCD59−) frequencies in 217 subjects from general population. The majority of subjects had a relatively low mutant frequencies (MFs) (average, 5.25 ± 3.6 × 10−6, median, 4.38 × 10−6, for all subjects), but with males having a significantly greater MFs (5.97 ± 4.0 × 10−6) than females (4.19 ± 2.5 ×10−6). There was no correlation between MFs and age. In addition, MFs showed no difference between smoker and nonsmoker, and also no association with smoke duration in male subjects. However, there was a significant correlation between cigarette‐pack‐years which indicated that the MF was only slightly elevated with the increase of cigarette‐pack‐years. Moreover, intraindividual variations were investigated in three volunteer subjects over 300 days, and the MFs were relatively stable and repeatable. Furthermore, a pilot study by using white blood cell (WBC) assay based on labeling with FLAER was performed in volunteer subjects. The MFs of FLAER‐deficient WBC (WBCFLAER−) and RBCCD59− were consistently elevated in two subjects. Our findings provide baseline data that will be helpful in designing further studies using the PIG‐A assay to monitor the genotoxic effects of carcinogens in human populations. Environ. Mol. Mutagen. 57:589–604, 2016. © 2016 Wiley Periodicals, Inc.
Introduction to ‘Technological infrastructure and international competitiveness’ by Christopher Freeman

This is an introduction to the publication for the first time of a 20-year-old draft paper by Christopher Freeman, the first paper that uses the concept 'national system of innovation'. The draft paper illustrates the role played by Christopher Freeman in building innovation research. This brief introduction gives the status of innovation system discourse and research, and relates it to what Freeman wrote more than 20 years ago. It challenges the idea that 'innovation system is not a theoretical concept', and shows that current innovation system analysis can still learn from Freeman when it comes to linking together trade policy and national innovation systems. Copyright 2004, Oxford University Press.
Social Inequalities in Perceived Health and the Use of Health Services in a Southern European Urban Area

People of lower social class have worse health and less access to health services and preventive care. This article describes social class inequalities in health status and use of services, both curative and preventive, in Barcelona, in a country with a national health service. The cross-sectional study uses information from the 1992 Barcelona Health Interview Survey. Social class was designated using an adaptation of the British Registrar General classification. The study variables measured health status, health services utilization, and preventive practices. Bivariate and multivariate analyses were used. Some 88 percent of men in social class I and 81 percent in class V had very good or good perceived health status. For women these figures were 85.2 and 57.6 percent, respectively. Chronic illness increased with lower social class. There were no social class differences in the frequency of physician visits during the two weeks prior to the interview among people with poor perceived health. Some 60.7 percent of women aged over 29 in social class I had periodic cervical smears, but only 32 percent of those in class V; the corresponding figures for mammography were 37.8 and 11.3 percent. The national health service has advantages in terms of access to health services, but more knowledge about the quality of these services is required. The study findings are sufficient to defend the undertaking of equitable health policies, especially in providing access to preventive care for the entire population.
Novel proton MR spectroscopy findings in adenylosuccinate lyase deficiency

Adenylosuccinate lyase (ADSL) deficiency is a rare inborn error of metabolism resulting in accumulation of metabolites including succinylaminoimidazole carboxamide riboside (SAICAr) and succinyladenosine (S‐Ado) in the brain and other tissues. Patients with ADSL have progressive psychomotor retardation, neonatal seizures, global developmental delay, hypotonia, and autistic features, although variable clinical manifestations may make the initial diagnosis challenging. Two cases of the severe form of the disease are reported here: an 18‐month‐old boy with global developmental delay, intractable neonatal seizures, progressive cerebral atrophy, and marked hypomyelination, and a 3‐month‐old girl presenting with microcephaly, neonatal seizures, and marked psychomotor retardation. In both patients in vivo proton magnetic resonance spectroscopy (MRS) showed the presence of S‐Ado signal at 8.3 ppm, consistent with a prior report. Interestingly, SAICAr signal was also detectable at 7.5 ppm in affected white matter, which has not been reported in vivo before. A novel splice‐site mutation, c.IVS12 + 1/G > C, in the ADSL gene was identified in the second patient. Our findings confirm the utility of in vivo proton MRS in suggesting a specific diagnosis of ADSL deficiency, and also demonstrate an additional in vivo resonance (7.5 ppm) of SAICAr in the cases of severe disease. J. Magn. Reson. Imaging 2013;37:974–980. © 2012 Wiley Periodicals, Inc.
On the Assessment of Financial Crises in the US Equity Market

This paper focuses on the development of the Index of Equity Market Pressure (IEMP) to characterize and to assess market turbulences. Similarities with existing risk measures result firstly from the method of financial crisis information extraction using financial and macroeconomic risk factors (Cf. Illing and Liu, 2003). The second affinity comes from the analogy of the IEMP with the Richter scale, measure of intensity of earthquake (Cf. Zumbach et al., 2000). However, in a first issue, we contribute to financial crises literature by proposing a new dynamic risk measure built from a factorial model mobilizing a robust and a dynamic estimation method respectively known as Iteratively Reweighted Least Squares and Kalman Filter. The first one helps us to select the significant risk factors by underweighting time series outliers whereas the second one allows us to measure time-varying betas more efficiently and to reconstruct a smoothed stock return trajectory. The final result gives us an asymmetric financial measure which assesses the intensity of negative rough returns. Based on monthly US market data from 1994 to 2009 period, we seek to compute objective and robust measures able to identify and characterize non-extreme and extreme event periods on stock index market.