Miracle-dz/UniFAR-FTUs huggingface dataset:
Muscular dystrophy: new challenges and review of the current clinical trials

Purpose of reviewWe provide a review of recent standards of care and therapeutic development in different forms of muscular dystrophies. This topic is relevant as the improved understanding of these disorders has not only led to a better definition of clinical course and to the development of standards of care for individual types of muscular dystrophies, but also culminated in different therapeutic approaches. Recent findingsRecent natural history studies have demonstrated the impact of new standards of care in different forms of muscular dystrophies, and identified areas of clinical management in which further developments are needed. The majority of the experimental studies are focused on Duchenne muscular dystrophy. Some of them target patients with specific mutations, such as antisense oligonucleotides, to induce exon skipping of specific mutations or drugs developed to allow read-through of nonsense mutations, whereas other therapies deal with secondary aspects of muscle degeneration, aiming, for example, at reducing inflammation or apoptosis, and may also be suitable for other forms of muscular dystrophies. SummaryThe advances in the field of muscular dystrophy have resulted in improved clinical course and survival. The encouraging results of early experimental studies could further improve these outcomes in the future.
Plagiarism detection in Chinese based on chunk and paragraph weight

Aiming at the Chinese academic paper plagiarism detection, proposed chunk based plagiarism detection algorithm with chunk extraction method based on character or word. Taken account of that different part of document has different importance, proposed two paragraph weight algorithms and defined three paragraph weight functions. The best chunk lengths are determined by experiments. Experiments show that using paragraph weight can enhance the detection effect.
Prevalence of Chronic Kidney Disease in Hypertensive Patients in Ghana

Chronic kidney disease (CKD) is common in tropical Africa although there are few data on the prevalence of this disorder. Therefore we initiated a multicenter screening study to identify the prevalence and staging of CKD in 712 patients with known hypertension in four polyclinics in Accra, Ghana. We measured estimated glomerular filtration rate by the six-variable modification of diet in renal disease equation and proteinuria by the protein/creatinine ratio. All the subjects studied were Ghanaian. Of the 712 patients studied, the median age was 59 years (range 19–90 years) and 560 (78.7%) of the patients were female. The mean duration of hypertension was 4 years (range 0.1–50). The overall prevalence of CKD was 46.9% (95% CI: 43.2–50.7%); 19.1% had CKD stages 1–2 and 27.8% had CKD stages 3–5. There was no difference in age between patients with or without CKD (p = 0.12). The overall prevalence of proteinuria was 28.9% (95% CI: 25.6–32.4%); 14.7% of subjects had preexisting diabetes mellitus and their prevalence of CKD (55%; 95% CI: 42.4–62.2) did not differ from those without diabetes (46%; 95% CI: 41.9–50.0, p = 0.133). CKD is common in hypertensive patients in Ghana, with a prevalence of 46.9%. This provides justification for the inclusion of this group in CKD screening programs in Ghana.
Optimization of [Amim]Cl Pretreatment Conditions for Maximum Glucose Recovery from Hybrid Pennisetum by Response Surface Methodology

Because of a complex chemical ultra-structure of lignocellulosic biomass, pretreatment is a necessary step for its conversion into bio-ethanol. In the present study, pretreatment conditions using the ionic liquid (IL) 1-allyl-3-methylimidazolium chloride ([Amim]Cl) were optimized for a relatively new model energy crop, hybrid Pennisetum (P. americanum × P. purpureum) to maximize the yield of fermentable sugars (glucose). The design of experiment programs employed a central composite design (CCD), with variables of temperature (102 to 187 °C), retention time (0.5 to 5.5 h), and solids loading (2 to 15 wt%). These factors were further optimized using response surface methodology (RSM). The proposed quadratic model to predict the glucose recovery from hybrid Pennisetum was verified by variance analysis (ANOVA). The model displayed high F and R2 values, indicating that it could be successfully used to identify the relationship among the independent variables studied. A maximum glucose recovery of 72.2% was found with temperature conditions of 139 °C, 2.97 h retention time, and 9.1 wt% solids loading.
Isolation and purification of a peptido-rhamnomannan from the yeast form of Sporothrix schenckii. Structural and immunochemical studies.

A peptido-rhamnomannan was isolated from the cells and culture medium of the human pathogenic fungus Sporothrix schenckii, grown in the yeast phase. It was purified by precipitation of its borate complex with hexadecyltrimethylammonium bromide and then by DEAE-Sephadex chromatography. The sugars in the polysaccharide were identified as d-mannose (50%) and l-rhamnose (33%). The amino acid composition of the peptide resembled that of the peptide moiety of other fungal peptido-polysaccharides. The polymer was almost entirely (91%) precipitated by concanavalin A and the ratio of mannose to rhamnose in the precipitate was unchanged, showing that the sugars are components of the same polymer. Methylation analysis and partial acid hydrolysis showed that the basic structure of the polysaccharide is a peptido-mannan to which numerous l-rhamnose residues are attached. About 60% of the rhamnose residues could be preferentially removed by mild acid hydrolysis. The peptido-rhamnomannan precipitated with the sera of individuals (3) with sporotrichosis. Sera from a small number of patients with other fungal diseases and from normal individuals did not precipitate within the sensitivity limits of the method. The specificity of two of the sera were examined in more detail by studying their cross-reactions with other fungal mannans.
Global surface temperature signals in pine ring‐width chronologies from southern monsoon Asia

We analyze Pinus ring width chronologies from three locations across monsoon Asia (Bhutan, India, and Thailand) where climate is dominated by the southwest monsoon in the boreal summer. We compare these records to global surface temperatures for the past 150 years, shifting the correlations through three seasonal averages: two seasons preceding the monsoon (Dec–Feb and Mar–May), and the monsoon season itself (Jun–Sep). Clear patterns emerge for each of the chronologies that highlight links to areas of known influence on the Asian monsoon: the Indian Ocean, the tropical eastern Pacific Ocean, and the high‐latitude Asian landmass. The Thai and Indian chronologies are from the same species (P. merkusii), and show a strong correlation with tropical Indian and Pacific Ocean bands. The Bhutan chronology (P. Wallichiana) is most strongly linked to climate over the north Pacific and Asian landmass. All of these correlations are strongest in seasons preceding the summer monsoon.
On the computational power of neural nets

This paper deals with finite networks which consist of interconnections of synchronously evolving processors. Each processor updates its state by applying a “sigmoidal” scalar nonlinearity to a linear combination of the previous states of all units. We prove that one may simulate all Turing Machines by rational nets. In particular, one can do this in linear time, and there is a net made up of about 1,000 processors which computes a universal partial-recursive function. Products (high order nets) are not required, contrary to what had been stated in the literature. Furthermore, we assert a similar theorem about non-deterministic Turing Machines. Consequences for undecidability and complexity issues about nets are discussed too.
An Efficient Robust Servo Design for Non-Minimum Phase Discrete-Time Systems with Unknown Matched/Mismatched Input Disturbances

This paper presents an efficient proportional-plus-integral (PI) current-output observer-based linear quadratic discrete tracker (LQDT) design methodology for the non-minimum-phase (NMP) discrete-time system with equal input and output number, for which the minimalized dynamic system contains the unmeasurable system state and unknown external matched/mismatched input disturbances. Illustrative examples are given to demonstrate the effectiveness of the proposed approach.
Who's at risk? Ethnic drinking cultures, foreign nativity, and problem drinking among Asian American young adults.

OBJECTIVE Despite the low overall prevalence of alcohol use among Asian Americans, rates of alcohol use disorder are high among Asian American young adults. The influence of ethnic drinking cultures on immigrants and their descendants has been overlooked in past research. We took an integrative approach to examine the influence of ethnic drinking culture, acculturation, and socioeconomic disparities on problem drinking among Asian American young adults. METHOD This study was a nationally representative sample of 854 Asian American young adults extracted from the Wave 4 National Longitudinal Study of Adolescent Health data. About 48% of the sample was female and 52% male. Several multiple logistic regression models were fitted. RESULTS Controlling for other covariates, two dimensions of ethnic drinking culture were associated with alcohol outcomes only for the foreign born: (a) detrimental drinking pattern with frequent drunkenness and alcohol-abuse symptoms and (b) drinking prevalence with alcohol-dependence symptoms. Financial hardship was a significant predictor of symptoms of alcohol abuse and dependence only for the U.S. born. Asian language use was protective against alcohol-abuse symptoms and alcohol-dependence symptoms for the foreign born. CONCLUSIONS Cultural and socioeconomic factors of problem drinking may be different for U.S.-and foreign-born Asian American young adults. Ethnic drinking cultures may significantly influence problem drinking of foreign-born Asian American young adults, independent of their acculturation into U.S. cultures. To inform effective interventions targeted at immigrants and their descendants, future research might further investigate the cultural and socioeconomic processes in immigrant communities that might significantly influence drinking.
Knowledge Management Systems for Higher Education Institutions : An Empirical Study of Success Factors

The number of KMS being implemented in higher education institutions (HEIs) has been increasing. However, there has been relatively little research to examine the factors that contribute to the success of KMS in HEIs. This study contributes to bridging this gap by examining the factors that influence the success of knowledge management systems (KMS) in higher education institutions (HEIs) by considering organizational and technological factors. The research model was based on DeLone and McLean IS success model, and modified to include organizational factors. The data was collected from 204 academicians in Malaysian public universities, and was confirmed of their reliability and validity using partial least square approach. It is hoped that the proposed model can be used as guidelines by stakeholders in implementing successful KMS in higher education.
De novo mutation in the GNAL gene causing seemingly sporadic dystonia in a Serbian patient

Mutations in GNAL (DYT25) have recently been established as the first confirmed cause of focal or segmental adult‐onset dystonia. Mutation carriers show craniocervical involvement; however, the GNAL mutational and phenotypic spectrum remain to be further characterized, and guidelines for diagnostic testing need to be established.
RFLP analysis of ribosomal DNA in sibling species of spider mite, genus Panonychus (Acari: Tetranychidae)

Ribosomal DNA (rDNA) restriction fragment length of spider mites was analysed in three sibling species of genus Panonychus (P. mod, P. citri and P. ulmi) and Tetranychus urticae. Seventy‐one fragments were detected by enhanced chemiluminescence (ECL) after Southern hybridization with two genomic rDNA probes of mouse. Percentages of shared fragments within species were larger than those among species, indicating that the variation of rDNAs was useful in phylo‐genetic studies of spider mite species. In a phenogram based on differences of the rDNA fragment length, the relationship among Panonychus species was compatible with the results of analysis based on the morphological characters.
Leptin deficiency and leptin gene mutations in obese children from Pakistan.

BACKGROUND Congenital leptin deficiency is a rare human genetic condition clinically characterized by hyperphagia and acute weight gain usually during the first postnatal year. The worldwide data on this disorder includes only 14 cases and four pathogenic mutations have been reported in the leptin gene. STUDY OBJECTIVE The objectives of this study were to measure serum leptin levels in obese children and to detect leptin gene mutations in those found to be leptin deficient. PATIENTS AND RESULTS A total of 25 obese children were recruited for the study. Leptin deficiency was detected in nine of them. Leptin gene sequencing identified mutations in homozygous state in all the leptin deficient children. Two cases carried novel mutations (c.481_482delCT and c.104_106delTCA) and each of the remaining seven the previously reported frameshift mutation (c.398delG). CONCLUSION The results suggest that leptin deficiency caused by mutations in the leptin gene may frequently be seen in obese Pakistani children from Central Punjab.
Racial Differences in the Incidence of Acute Low Back Pain in United States Military Service Members

Study Design. An epidemiological study. Objective. To determine the effect of race on the incidence of acute low back pain, resulting in a health care encounter in active duty military service members. Summary of Background Data. Although racial differences in the incidence of low back pain have been documented in previous studies, currently no consensus exists on the relative risk between these groups. Methods. A query was performed using the Armed Forces Health Surveillance Center database for the International Classification of Diseases, Ninth Revision code for low back pain (724.20). A total of 12,399,276 person-years of data were analyzed and stratified by age, race, and sex. Incidence rates were calculated and compared using the multivariate Poisson regression analysis. Results. A total of 467,950 cases of low back pain resulted in a visit to a health care provider in our population, with an overall incidence rate of 37.74 per 1000 person-years. Asians/Pacific Islanders had the lowest incidence rate of 30.7 and blacks had the highest with 43.7. Female sex and older age were also significant risk factors but with significantly different effect sizes between racial groups. Native Americans/Alaskan Natives demonstrated the greatest effect of age on low back pain incidence rates, with a 126% increase between the youngest and oldest age groups compared with a 36% difference in whites. Conclusion. Race, sex, and age were all found to be significant risk factors for acute low back pain. The highest rates were identified in blacks followed by whites, Hispanics, and American Indian/Alaskan Native, and the lowest rates were identified in Asians/Pacific Islanders. Significant differences in the effect of sex and age were identified between the different racial groups.
Deletions in the tRNA(Lys) primer-binding site of human immunodeficiency virus type 1 identify essential regions for reverse transcription

The initiation of human immunodeficiency virus type 1 (HIV-1) reverse transcription occurs by the extension of a tRNA primer bound near the 5' end of the genomic RNA at a position termed the primer-binding site (PBS). The PBS is an 18-nucleotide region of the HIV-1 genome complementary to cellular tRNA(Lys). To further investigate the sequence requirements for the PBS in reverse transcription, deletions in the PBS were created and subcloned into a plasmid containing the infectious HIV-1 proviral genome. The mutations deleted the entire PBS (delta PBS) or the first 9 (delta 1-9), the second 9 (delta 10-18), or 12 (delta 7-18) nucleotides of the PBS. An additional mutation in the PBS was created in which the second nine nucleotides were deleted and nine additional nucleotides were substituted [Lys(1-9)]. The transfection of plasmids containing the wild-type or mutant proviral genomes into tissue culture cells resulted in expression of the HIV-1 gag and env gene products, as determined by immunoprecipitation using sera from AIDS patients. HIV-1 virus was released from the transfected cells, as determined by analysis of the supernatants for reverse transcriptase activity. The infectivity of the viruses derived from the transfection was examined by coculture experiments with SupT1 cells, which support high-level replication of HIV-1. The transfection of plasmids containing HIV-1 proviral genomes with the delta PBS and PBS (delta 1-9) mutations did not produce infectious virus. In contrast, the HIV-1 proviral genomes with the delta 10-18, delta 7-18, and Lys(1-9) mutations in the PBS produced infectious virus upon transfection, although the kinetics of appearance was significantly delayed for the mutant viruses compared with the wild type. To further explore the nature of this defect, the PBS region from integrated proviral genomes was amplified by polymerase chain reaction and individual DNA products were subcloned into M13mp19, followed by a sequence analysis of the PBS region from individual M13 phage clones. In each of the PBS regions examined, the 18-nucleotide PBS complementary to tRNA(Lys) was present. However, nucleotide deletions and insertions were found 3' to the PBS from the samples derived from the transfection of plasmids containing mutant proviral genomes. Upon reinfection, the revertant viruses maintained the deletions 3' to the PBS and had kinetics of replication similar to that of the wild-type virus. (ABSTRACT TRUNCATED AT 400 WORDS)
Podoplanin expression by cancer associated fibroblasts predicts poor prognosis of lung adenocarcinoma

Recent studies have reported increased podoplanin expression by cancer cells and stromal cells, but little is known about its expression and biological significance in adenocarcinoma of the lung. We examined podoplanin expression by both cancer cells and stromal cells in 177 consecutive lung adenocarcinoma cases and analyzed relations between podoplanin expression and both clinicopathological factors and outcome. Podoplanin expression was observed on the apical membrane of the cancer cells in only 9 of the 177 (5.1%) cases. By contrast, cancer‐associated fibroblasts (CAFs) were found to express podoplanin in 54 cases (30.5%). Podoplanin (+) CAFs were found only in invasive adenocarcinoma and none were found in noninvasive adenocarcinoma. Conventional prognostic factors were significantly correlated with podoplanin expression by CAFs. The univariate analyses and log‐rank test showed that podoplanin expression was significantly associated with shorter survival time (p < 0.001 and p < 0.001, respectively). We divided the cases into 3 groups according grade based on the proportion of CAFs expressing podoplanin [a grade 0 group (n = 123), a grade 1 group (n = 36) and a grade 2 group (n = 18)]. The result showed that conventional prognostic factors were significantly correlated with the grade of podoplanin expression by CAFs. Furthermore, the grade 2 group tended to have a shorter survival time than the grade 1 group (p = 0.092). The results of this study highlight the importance of podoplanin expression by CAFs and provide new insights into the biology of the cancer microenvironment in adenocarcinoma of the lung. © 2008 Wiley‐Liss, Inc.
Transient and Stable Knockdown of the Integrase Cofactor LEDGF/p75 Reveals Its Role in the Replication Cycle of Human Immunodeficiency Virus

ABSTRACT After identifying the interaction between the transcriptional coactivator lens epithelium-derived growth factor (LEDGF/p75) and the human immunodeficiency virus type 1 (HIV-1) integrase (IN), we have now investigated the role of LEDGF/p75 during HIV replication. Transient small interfering RNA-mediated knockdown of LEDGF/p75 in HeLaP4 cells resulted in a three- to fivefold inhibition of HIV-1 (strain NL4.3) replication. Quantitative PCR was used to pinpoint the replication block to the integration step. Next, polyclonal and monoclonal HeLaP4-derived cell lines were selected with a stable knockdown of LEDGF/p75 mediated by a lentiviral vector (lentivector) encoding a short hairpin RNA (shRNA) targeting this protein. Cell lines stably transduced with a lentivector encoding an unrelated hairpin or a double-mismatch hairpin served as controls. Again, a two- to fourfold reduction of HIV-1 replication was observed. The extent of LEDGF/p75 knockdown closely correlated with the reduction of HIV-1 replication. After the back-complementation of LEDGF/p75 in the poly- and monoclonal knockdown cell lines using an shRNA-resistant expression plasmid, viral replication was restored to nearly wild-type levels. The Q168A mutation in integrase has been shown to interfere with the interaction with LEDGF/p75 without reducing the enzymatic activity. Transduction by HIV-1-derived lentivectors carrying the Q168A IN mutant was severely hampered, pointing again to a requirement for LEDGF/p75. Altogether, our data validate LEDGF/p75 as an important cellular cofactor for HIV integration and as a potential target for antiviral drug development.
Caenorhabditis elegans as an Environmental Monitor Using DNA Microarray Analysis

Abstract: In order to assist in the identification of possible endocrine disrupting chemicals (EDC) in groundwater, we are developing Caenorhabolitis elegans as a high throughput bioassay system in which responses to EDC may be detected by gene expression using DNA microarray analysis. As a first step we examined gene expression patterns and vitellogenin responses of this organism to vertebrate steroids, in liquid culture. Western blotting showed the expected number and size of vitellogenin translation products after estrogen exposure. At 10−9 M, vitellogenin decreased, but at 10−7 and 10−5, vitellogenin was increased. Testosterone (10−5 M) increased the synthesis of vitellogenin, but progesterone‐treated cultures (10−5 M) had less vitellogenin. Using DNA microarray analysis, we examined the pattern of gene expression after progesterone (10−5, 10−7, and 10−9 M), estrogen (10−5 M), and testosterone (10−9 M) exposure, with special attention to the traditional biomarker genes used in environmental studies [vitellogenin, cytochrome P450 (CYP), glutathione s‐transferase (GST), metallothionein (MT), and heat shock proteins (HSP)]. GST and P450 genes were affected by estrogen (10−5 M) and progesterone (10−5 and 10−7 M) treatments. For vitellogenin genes, estrogen treatment (10−5 M) caused overexpression of the vit‐2 and vit‐6 genes (2.68 and 3.25 times, respectively). After progesterone treatment (10−7 M), the vit‐5 and vit‐6 were down‐regulated and vit‐1 up‐regulated (3.59‐fold). Concentrations of testosterone and progesterone at 10−9 M did not influence the expression of the vit, CYP, or GST genes. Although the analysis is incomplete, and low doses and combinations of EDC need to be tested, these preliminary results indicate C. elegans may be a useful laboratory and field model for screening EDC.