Miracle-dz/UniFAR-FTUs huggingface dataset:
Prognostic value of minimal residual disease quantification before allogeneic stem-cell transplantation in relapsed childhood acute lymphoblastic leukemia: the ALL-REZ BFM Study Group.

PURPOSE Minimal residual disease (MRD) before allogeneic stem-cell transplantation was shown to predict outcome in children with relapsed acute lymphoblastic leukemia (ALL) in retrospective analysis. To verify this, the Acute Lymphoblastic Leukemia Relapse Berlin-Frankfurt-Münster (ALL-REZ BFM) Study Group conducted a prospective trial. PATIENTS AND METHODS Between March 1999 and July 2005, 91 children with relapsed ALL treated according to the ALL-REZ BFM 96 or 2002 protocols and receiving stem-cell transplantation in >or= second remission were enrolled. MRD quantification was performed by real-time polymerase chain reaction using T-cell receptor and immunoglobulin gene rearrangements. RESULTS Probability of event-free survival (pEFS) and cumulative incidence of relapse (CIR) in 45 patients with MRD >or= 10(-4) leukemic cells was 0.27 and 0.57 compared with 0.60 and 0.13 in 46 patients with MRD less than 10(-4) leukemic cells (EFS, P = .004; CIR, P < .001). Intermediate-risk patients (strategic group S1) with MRD >or= 10(-4) leukemic cells (n = 14) had a pEFS of 0.20 and CIR of 0.73, whereas patients with MRD less than 10(-4) leukemic cells (n = 21) had a pEFS of 0.68 and CIR of 0.09 (EFS, P = .020; CIR, P < .001). High-risk patients (S3/4, third complete remission) who received transplantation with an MRD load of less than 10(-4) leukemic cells (n = 25) showed a pEFS and CRI of 0.53 and 0.18, respectively. In contrast, pEFS and CRI were 0.30 and 0.50 in patients who received transplantation with an MRD load of >or= 10(-4) leukemic cells. Multivariate Cox regression analysis revealed MRD as the only independent parameter predictive for EFS (P = .006). CONCLUSION MRD is an important predictor for post-transplantation outcome. As a result, new strategies with modified stem-cell transplantation procedures will be evaluated in ALL-BFM trials.
Low-Complexity Compressive Sensing Detection for Spatial Modulation in Large-Scale Multiple Access Channels

In this paper, we propose a detector, based on the compressive sensing (CS) principles, for multiple-access spatial modulation (SM) channels with a large-scale antenna base station (BS). Particularly, we exploit the use of a large number of antennas at the BSs and the structure and sparsity of the SM transmitted signals to improve the performance of conventional detection algorithms. Based on the above, we design a CS-based detector that allows the reduction of the signal processing load at the BSs particularly pronounced for SM in large-scale multiple-input-multiple-output (MIMO) systems. We further carry out analytical performance and complexity studies of the proposed scheme to evaluate its usefulness. The theoretical and simulation results presented in this paper show that the proposed strategy constitutes a low-complexity alternative to significantly improve the system's energy efficiency against conventional MIMO detection in the multiple-access channel.
Therapeutic strategies for spinal muscular atrophy: SMN and beyond

ABSTRACT Spinal muscular atrophy (SMA) is a devastating neuromuscular disorder characterized by loss of motor neurons and muscle atrophy, generally presenting in childhood. SMA is caused by low levels of the survival motor neuron protein (SMN) due to inactivating mutations in the encoding gene SMN1. A second duplicated gene, SMN2, produces very little but sufficient functional protein for survival. Therapeutic strategies to increase SMN are in clinical trials, and the first SMN2-directed antisense oligonucleotide (ASO) therapy has recently been licensed. However, several factors suggest that complementary strategies may be needed for the long-term maintenance of neuromuscular and other functions in SMA patients. Pre-clinical SMA models demonstrate that the requirement for SMN protein is highest when the structural connections of the neuromuscular system are being established, from late fetal life throughout infancy. Augmenting SMN may not address the slow neurodegenerative process underlying progressive functional decline beyond childhood in less severe types of SMA. Furthermore, individuals receiving SMN-based treatments may be vulnerable to delayed symptoms if rescue of the neuromuscular system is incomplete. Finally, a large number of older patients living with SMA do not fulfill the present criteria for inclusion in gene therapy and ASO clinical trials, and may not benefit from SMN-inducing treatments. Therefore, a comprehensive whole-lifespan approach to SMA therapy is required that includes both SMN-dependent and SMN-independent strategies that treat the CNS and periphery. Here, we review the range of non-SMN pathways implicated in SMA pathophysiology and discuss how various model systems can serve as valuable tools for SMA drug discovery. Summary: Translational research for spinal muscular atrophy (SMA) should address the development of non-CNS and survival motor neuron (SMN)-independent therapeutic approaches to complement and enhance the benefits of CNS-directed and SMN-dependent therapies.
Epidemiology of Crohn's disease in Israel: a survey of Israeli kibbutz settlements

Objective:The prevalence of Crohn's disease ranges from 10 to 70 cases per 100,000 population, and is 3–8 times more common among Jews. However, this excess risk is not evident in the Jewish population of Israel. Recently we have described a significant increase in the prevalence and incidence of Crohn's disease in the south of Israel. The aim of this study was to confirm this trend in a stable population found in communal (kibbutz) settlements.Methods:We repeated a community-based survey in 124,400 kibbutz residents, 10 yr after our first study. This population represents 5% of the Jewish population of Israel. All Crohn's disease patients were located by contacting the kibbutz clinics of the 269 kibbutz settlements (100% compliance). Data was updated to December 31st, 1997, which was designated the point prevalence date, and included information on gender, age, origin, education, profession, extent of the inflammatory process, clinical spectrum of the disease, therapy, complications of the disease, and treatment. The average annual incidence for the 10 yr was calculated from the prevalence data. Only cases with a definite diagnosis of Crohn's disease made in a recognized gastroenterology unit were accepted into the study.Results:There were 81 confirmed cases of Crohn's disease and the prevalence rate rose from 25.5/100,000 in 1987 to 65.1/100,000 in 1997 (p < 0.001). The mean annual incidence rate for this period (1987–1997) is 5.0/100,000/yr. Prevalence rates were higher in women than men, and in those born in Israel or Europe/America than in Asia/Africa. The mean age at presentation of the disease was lower in 1997 than in 1987, 37.4 ± 17.0 and 45.0 ± 17.0 yr, respectively (p= 0.041). Prevalence was highest in men with >16 yr of education, and in women with 11–12 yr education, 119.7 and 100.3/100,000, respectively.Conclusion:During the decade 1987–1997, the prevalence of Crohn's disease has increased in Israel and is approaching the rates in Europe and America.
The antioxidant level of Alaska’s wild berries: high, higher and highest

Background In the last few years, antioxidants have become the stars of the nutritional world. Antioxidants are important in terms of their ability to protect against oxidative cell damage that can lead to conditions, such as Alzheimer's disease, cancer and heart disease – conditions also linked with chronic inflammation. The antioxidant and anti-inflammatory effects of Alaska's wild berries may have the potential to help prevent these diseases. Objective To discover the antioxidant levels of Alaska wild berries and the ways these antioxidant levels translate when preservation methods are applied to the berry. Design This research centred on both the raw berries and products made from the berries. In the first year, a variety of wild berries were tested to discover their oxygen radical absorption capacity (ORAC) in the raw berries. The second level of the research project processed 4 different berries – blueberries, lingonberries, salmonberries, highbush cranberries – into 8 or 9 products made from these berries. The products were tested for both ORAC as well as specific antioxidants. Results The Alaska wild berries collected and tested in the first experiment ranged from 3 to 5 times higher in ORAC value than cultivated berries from the lower 48 states. For instance, cultivated blueberries have an ORAC scale of 30. Alaska wild dwarf blueberries measure 85. This is also higher than lower 48 wild blueberries, which had a score of 61. All of the Alaskan berries tested have a level of antioxidant considered nutritionally valuable, ranging from 19 for watermelon berries to 206 for lingonberries on the ORAC scale. With the processed products made from 4 Alaska wild berries, one of the unexpected outcomes of the research was that the berries continued to have levels of antioxidants considered high, despite the effects of commonly used heat-processing techniques. When berries were dehydrated, per gram ORAC values increased. Conclusion Alaska wild berries have extraordinarily high antioxidant levels. Though cooking lowered the antioxidant level, and adding ingredients such as sugar diluted the antioxidant concentration, products made from berries are high sources of antioxidants.
Brachytelephalangic dwarfism due to the loss of ARSE and SHOX genes resulting from an X;Y translocation

Here we report an 8‐year‐old male patient who had mesomelic shortening of forearms and legs, brachytelephalangia and ichthyotic skin lesions. Chromosomal analysis showed an X;Y translocation involving the short arm of the X chromosome (Xp). Fluorescence in situ hybridization (FISH) and molecular studies localized the breakpoints on Xp22.3 in the immediate vicinity of the KAL gene demonstrating deletions of steroid sulfatase (STS), arylsulfatase E (ARSE), and short stature homeo box (SHOX) genes. It was suspected that the patient was suffering from chondrodysplasia punctata because of a loss of the arylsulfatase E (ARSE) gene. However, no stippled epiphyses were to be seen in the neonatal radiograph. Interestingly, this patient is the first case with a proven loss of the ARSE gene without chondrodysplasia punctata, assuming that chondrodysplasia punctata is not an obligatory sign of ARSE gene loss. Brachytelephalangia was the only result of ARSE gene deletion in this case. The patient's mother also had dwarfism and showed Madelung deformity of the forearms. She was detected as a carrier of the same aberrant X chromosome. The male patient did not show Madelung deformity, demonstrating that Lerri–Weill syndrome phenotype may be still incomplete in children with SHOX gene deletion. The wide clinical spectrum in the male and the Leri–Weill phenotype in his mother are the results of both a deletion involving several sulfatase genes in Xp22.3 and the SHOX gene located in the pseudoautosomal region. Nevertheless, there is no explanation for the absence of chondrodysplasia punctata despite the total loss of the ARSE gene. Further studies are necessary to investigate genotype/phenotype correlation in cases with translocations or microdeletions on Xp22.3, including the ARSE and the SHOX gene loci.
Climatological characteristics of cloud distribution and planetary boundary layer structure in Jakarta, Indonesia revealed by lidar observation

For two years we observed the vertical distribution of clouds and aerosols in Jakarta, Indonesia, with ground‐based Mie scattering lidars. A histogram of the cloud base height shows a notable maximum at an altitude of approximately 5 km, especially in the wet season. The planetary boundary layer height we inferred from the upper boundary of the aerosol layer was approximately 1.5–2 km in the wet season and 2–3 km in the dry season.
Spatial Modeling of Nitrifier Microhabitats in Soil

Soil bacteria function in the three-dimensional space in heterogeneous soil complex and their activities depend in part on encountering substrates at the microbial scale. The bacterial density per gram of soil, which is generally measured, does not indicate if bacteria are all in the same location or spread throughout the soil complex. We characterized spatial distribution for how dispersed or aggregated nitrifiers (NH + 4 and NO - 2 oxidizers) were at a submillimeter scale. The spatial approach was based on the relationship, obtained experimentally, between the percentage of microsamples (50-500 μm diam.) harboring nitrifiers and the volume of the microsamples. The smallest sample size (50-μm diam.) was considered as an approximation of microhabitat. The simulated spatial pattern of NO - 2 oxidizer microhabitats in soil were compared with experimental data. The simulated pattern of NO - 2 oxidizer distribution suggested that microhabitats averaged seven NO - 2 oxidizers and occurred in preferentially colonized patches that had about a 250-μm diam. These were randomly distributed and occupied 5.5% of the soil volume. They were functionally connected through microporosity and hence diffusion processes probably controlled the spatial distribution of nirifiers. The nitrifier spatial pattern enabled efficient nitrification because NH + 4 and NO - 2 oxidizers were near one another. The results showed the potential of our method to study spatial distribution of bacteria at the microhabitat scale.
Temporally Resolved Imaging of Jet-Type Dielectric Barrier Discharge Using He and Ar/Acetone Crossed Gas Flows

We report on temporally resolved images in a jet-type dielectric barrier discharge with two crossed gas flows obtained by a spectrally filtered intensified charge-coupled device (ICCD) camera. The crossed-flow configuration of He and Ar gases and a small acetone impurity added into the Ar flow simulated a discharge configuration and an organic precursor used for the practical application of material processing, respectively. From the ICCD images, streamer and transient glow discharges could be distinguished between the electrode and the substrate. Filtered emission from excited N2+, CH, C2, He, and Ar also revealed the excitation and decomposition processes in the discharge.
AN EVALUATION OF WEEKLY AND MONTHLY TIME SERIES FORECASTS OF MUNICIPAL WATER USE

: A cascade model for forecasting municipal water use one week or one month ahead, conditioned on rainfall estimates, is presented and evaluated. The model comprises four components: long term trend, seasonal cycle, autocorrelation and correlation with rainfall. The increased forecast accuracy obtained by the addition of each component is evaluated. The City of Deerfield Beach, Florida, is used as the application example with the calibration period from 1976–1980 and the forecast period the drought year of 1981. Forecast accuracy is measured by the average absolute relative error (AARE, the average absolute value of the difference between actual and forecasted use, divided by the actual use). A benchmark forecast is calculated by assuming that water use for a given week or month in 1981 is the same as the average for the corresponding period from 1976 to 1980. This method produces an AARE of 14.6 percent for one step ahead forecasts of monthly data and 15.8 percent for weekly data. A cascade model using trend, seasonality and autocorrelation produces forecasts with AARE of about 12 percent for both monthly and weekly data while adding a linear relationship of water use and rainfall reduces the AARE to 8 percent in both cases if it is assumed that rainfall is known during the forecast period. Simple rainfall predictions do not increase the forecast accuracy for water use so the major utility of relating water use and rainfall lies in forecasting various possible water use sequences conditioned on sequences of historical rainfall data.
RNA Sequencing of Laser-Capture Microdissected Compartments of the Maize Kernel Identifies Regulatory Modules Associated with Endosperm Cell Differentiation[OPEN]

RNA profiling of maize kernel compartments revealed coexpression modules for each major cell type in the endosperm, including a module regulating differentiation of the basal endosperm transfer layer. Endosperm is an absorptive structure that supports embryo development or seedling germination in angiosperms. The endosperm of cereals is a main source of food, feed, and industrial raw materials worldwide. However, the genetic networks that regulate endosperm cell differentiation remain largely unclear. As a first step toward characterizing these networks, we profiled the mRNAs in five major cell types of the differentiating endosperm and in the embryo and four maternal compartments of the maize (Zea mays) kernel. Comparisons of these mRNA populations revealed the diverged gene expression programs between filial and maternal compartments and an unexpected close correlation between embryo and the aleurone layer of endosperm. Gene coexpression network analysis identified coexpression modules associated with single or multiple kernel compartments including modules for the endosperm cell types, some of which showed enrichment of previously identified temporally activated and/or imprinted genes. Detailed analyses of a coexpression module highly correlated with the basal endosperm transfer layer (BETL) identified a regulatory module activated by MRP-1, a regulator of BETL differentiation and function. These results provide a high-resolution atlas of gene activity in the compartments of the maize kernel and help to uncover the regulatory modules associated with the differentiation of the major endosperm cell types.
AN UPPER BOUND ON ALGEBRAIC CONNECTIVITY OF GRAPHS WITH MANY CUTPOINTS

Let G be a graph on n vertices which has k cutpoints. A tight upper bound on the algebraic connectivity of G in terms of n and k for the case that k>n /2 is provided; the graphs which yield equality in the bound are also characterized. This completes an investigation initiated by the author in a previous paper, which dealt with the corresponding problem for the case that k ≤ n/2. G). Finally, if G has vertex connectivity c ≤ n − 2, then α(G) ≤ c . I n particular, if G has a cutpoint - that is, a vertex whose deletion (along with all edges incident with it) yields a disconnected graph - then we see that α(G) ≤ 1. Motivated by this last observation, Kirkland (7) posed the following problem: if G is a graph on n vertices which has k cutpoints, find an attainable upper bound on α(G). In (7), such a bound is constructed for the case that 1 ≤ k ≤ n/2, and the graphs attaining the bound are characterized. The present paper is a continuation of the work in (7); here we give an attainable upper bound on α(G )w henn/2 <k ≤ n−2, and explicitly describe the equality case. The technique used in this paper relies on the analysis of the various connected components which arise from the deletion of a cutpoint. We now briefly outline that technique. Suppose that G is a connected graph and that v is a cutpoint of G.Th e components at v are just the connected components of G−v, the (disconnected) graph ∗ Received by the editors on 2 November 2000. Accepted for publication on 19 May 2001. Handling
A Meta-Relational Approach for the Definition and Management of Hybrid Learning Objects

Electronic learning objects (LOs) are commonly conceived of as digital units of information used for teaching and learning. To facilitate their classification for pedagogical planning and retrieval purposes, LOs are complemented with metadata (e.g., the author). These metadata are usually restricted by a set of predetermined tags to which the classification schema must conform (e.g., IEEE LOM). In our experience, certain complex LOs need to be complemented with different types of domain-dependent information for their pedagogical planning and retrieval: (i) classification metadata for enhancing contextualisation, search and retrieval (e.g., the tagged structure of an archaeological site where an archaeological object has been found) and (ii) additional data that can enrich the LO (e.g., the weight and other dimensions of an archaeological artifact described in a podcast). We refer to LOs enhanced with domain-dependent information as hybrid learning objects (HLOs). However, most learning object repositories (LORs) only permit a predetermined, fixed set of metadata attributes to be used in the classification of LOs. This rigidity is inappropriate when domain-dependent information schemas are used for the browsing, retrieval, and domain-specific pedagogical sequencing of HLOs. Thus, custom software applications are needed to manage LOs that must be tagged with information belonging to specific domains. This paper presents a theoretical approach that permits the use of a single LOR for classifying and enriching LOs according to domain-dependent information schemas, which can be dynamically changed after their definition. The key issue in our approach is the presence of a meta-relational model for the dynamic definition of specific domain-dependent relational database schemas used for classifying and enriching LOs.
Subword scheme for keyword search

Keyword search (KWS) is an important application of spoken language technology. The technique of Large Vocabulary Continuous Speech Recognition (LVCSR) is playing an important role in KWS system. However, for a language with large vocabulary and relatively insufficient text corpus, the vocabulary size keeps going up very quickly with the increasing amount of text, as we observed in Tamil. This brings difficulty in training a reliable language model, which may undermine KWS performance. Subword unit has been successfully employed in KWS system to handle out-of-vocabulary (OOV) problem. Inspired by this, we propose a novel subword scheme from the perspective of pronunciation to alleviate the large vocabulary problem. We find that the subword-based system outperforms our best word-based system on Tamil conversational telephone speech. The experiment of system combination shows that, over the best word-based system, a single subword-based system contains more complementary information than the total of that of the other three word-based systems.
ERK and β-Arrestin Interaction

β-Arrestin, a signal adaptor protein, mediates intracellular signal transductions through protein-protein interactions by bringing two or more proteins in proximity. Extracellular signal-regulated kinase (ERK), a protein kinase in the family of mitogen-activated protein kinases (MAPKs), is involved in various receptor signal pathways. Interaction of ERK with β-arrestin or formation of ERK/β-arrestin signal complex occurs in response to activation of a variety of cell surface receptors. The ERK/β-arrestin signal complex may be a common transducer to converge a variety of extracellular stimuli to similar downstream intracellular signaling pathways. By using a cell-based protein-protein interaction LinkLight assay technology, we demonstrate a direct interaction between ERK and β-arrestin in response to extracellular stimuli, which can be sensitively and quantitatively monitored. Activations of G protein–coupled receptors (GPCRs), receptor tyrosine kinases (RTKs), and cytokine receptors promote formation of the ERK/β-arrestin signal complex. Our data indicate that the ERK/β-arrestin signal complex is a common transducer that participates in a variety of receptor signaling pathways. Furthermore, we demonstrate that receptor antagonists or kinase inhibitors can block the agonist-induced ERK and β-arrestin interaction. Thus, the ERK/β-arrestin interaction assay is useful for screening of new receptor modulators.