Miracle-dz/UniFAR-FTUs huggingface dataset:
Hematopoietic Stem-Cell Transplantation in Globoid-Cell Leukodystrophy

A BSTRACT Background Globoid-cell leukodystrophy is caused by a deficiency of galactocerebrosidase, which results in progressive central nervous system deterioration. We investigated whether allogeneic hematopoietic stem-cell transplantation can provide a source of leukocyte galactocerebrosidase and thereby prevent the decline of central nervous system function in patients with the disease. Methods Five children with globoid-cell leukodystrophy (one with the infantile type and four with lateonset disease) were treated with allogeneic hematopoietic stem-cell transplantation. Measurement of leukocyte galactocerebrosidase levels, neurologic examinations, neuropsychological tests, magnetic resonance imaging of the central nervous system, cerebrospinal fluid protein assays, and neurophysiologic measurements were performed before and after transplantation, with follow-up ranging from one to nine years. Results Engraftment of donor-derived hematopoietic cells occurred in all patients and was followed by restoration of normal leukocyte galactocerebrosidase levels. In the four patients with late-onset disease, the central nervous system deterioration was reversed, and in the patient with the infantile form of the disease, signs and symptoms have not appeared. Magnetic resonance imaging showed a decrease in signal intensity in the three patients with late-onset disease who were assessed both before and after transplantation. Abnormalities in cerebrospinal fluid total protein levels were corrected in three patients with late-onset disease and substantially reduced in the patient with the infantile form. Conclusions Central nervous system manifestations of globoid-cell leukodystrophy can be reversed by allogeneic hematopoietic stem-cell transplantation. (N Engl J Med 1998;338:1119-26.)
Effects and mechanisms of Bacillus thuringiensis crystal toxins for mosquito larvae

Bacillus thuringiensis is a Gram‐positive aerobic bacterium that produces insecticidal crystalline inclusions during sporulation phases of the mother cell. The virulence factor, known as parasporal crystals, is composed of Cry and Cyt toxins. Most Cry toxins display a common 3‐domain topology. Cry toxins exert intoxication through toxin activation, receptor binding and pore formation in a suitable larval gut environment. The mosquitocidal toxins of Bt subsp. israelensis (Bti) were found to be highly active against mosquito larvae and are widely used for vector control. Bt subsp. jegathesan is another strain which possesses high potency against broad range of mosquito larvae. The present review summarizes characterized receptors for Cry toxins in mosquito larvae, and will also discuss the diversity and effects of 3‐D mosquitocidal Cry toxin and the ongoing research for Cry toxin mechanisms generated from investigations of lepidopteran and dipteran larvae.
Identification of the Apoptosis Activation Cascade Induced in Mammary Carcinomas by Energy Restriction

Energy restriction (ER) inhibits mammary carcinogenesis and results in a marked reduction in tumor size, effects likely to be explained by ER-mediated induction of apoptosis. The goal of this study was to investigate the molecular mechanism(s) accounting for apoptosis induction. To do this, chemically induced mammary carcinomas were evaluated from rats that were ad libitum fed (control), 40% ER, or 40% ER but energy repleted for 7 days before study termination (ER-REP); the ER-REP group permitted the determination of the reversibility of ER-mediated effects. Cleaved products of poly(ADP-ribose) polymerase 1 were elevated by ER (P < 0.025) providing biochemical evidence of apoptosis induction. cDNA microarray analysis identified the Bcl-2, CARD, and IAP functional gene groupings as being involved in apoptosis induction. Consistent with the microarray data, the activities of caspases 9 and 3 were observed to be ∼2-fold higher in carcinomas from ER rats (P ≤ 0.01), whereas caspase 8 activity was similar in carcinomas from all three of the groups. This evidence that ER-induced apoptosis mediated by the mitochondrial pathway was additionally supported by the finding that levels of Bcl-2, Bcl-xl, and XIAP protein were significantly lower (P < 0.01), and levels of Bax and Apaf-1 were elevated (P < 0.02) in ER carcinomas versus those carcinomas from control or ER-REP rats. Additional studies revealed that Akt phosphorylation (activation) was reduced in mammary carcinomas from ER rats. Thus, it appears that ER induces apoptosis in mammary carcinomas via a cell survival factor-dependent pathway.
Sustainable Yield in Theory and Practice: Bridging Scientific and Mainstream Vernacular

Groundwater is a vital resource in California, and the concept of “sustainable yield” is an attempt to determine a metric that can ensure the long‐term resilience of groundwater systems. However, its meaning is ambiguous and quantification is challenging. To provide insight into developing a working definition that encompasses the inherent uncertainty and complexity of the term, this paper examines how sustainable yield in groundwater is interpreted by (1) scientists, (2) the courts in groundwater adjudications, (3) state agencies, and (4) local water practitioners. Through qualitative interviews, this paper identifies problems that local water agencies in the state encounter in engaging with sustainable yield as they incorporate the term in groundwater management practices. The authors recommend that any definitions make explicit the human dimensions of, and assumptions embedded in, the use of these terms in groundwater management practices, and they point to the value of participation in this process.
Review: The Immunomodulatory Roles of Macrophages at the Maternal—Fetal Interface

Macrophages are versatile cells that play a central role in innate and adaptive immunity and participate in a wide variety of biological processes. In the uterine decidua, macrophages represent a major leukocyte subset throughout pregnancy. Here, decidual macrophages exert an immunosuppressive phenotype characterized by abundant production of interleukin (IL)-10 and indoleamine 2,3-dioxygenase activity. Their polarized cytokine secretion pattern has recently been classified as an M2 phenotype. These features of decidual macrophages favor maternal immune tolerance to semiallogenic fetus. In addition, macrophages cooperate with trophoblast cells during the early stages of human pregnancy to support uterine vasculature remodeling by removing apoptotic cells and through the production of proteases that degrade the extracellular matrix. In the peripartum period, macrophages also participate in the regulation of cervical ripening and the initiation of parturition through the production of proinflammatory cytokines and prostaglandin E2 (PGE2). Aberrant activity of uterine macrophages is linked to the pathogenesis of preeclampsia and preterm delivery. Here, we review the immunomodulatory roles of decidual macrophages during pregnancy.
Geographic variations in the frequency of thyroid disorders and thyroid peroxidase antibodies in persons without former thyroid disease within Germany.

OBJECTIVE To investigate regional variations in the frequency of undiagnosed thyroid disorders among 25- to 88-year-old men and women in two communities in the northeast and the south of Germany. In addition, autoantibodies to thyroperoxidase (TPO-Abs) and urinary iodine excretion were determined. METHODS Two population-based surveys of men and women using a common standardized protocol, the Study of Health in Pomerania (SHIP) in the northeast of Germany (2505 participants) and the Kooperative Gesundheitsforschung in der Region Augsburg (KORA) in the south of Germany (2316 participants), were compared with regard to the frequency of undiagnosed thyroid disorders. RESULTS Compared with the northeast of Germany, urinary iodine excretion and serum thyroid-stimulating hormone (TSH) levels were significantly higher in the south. The median urinary iodine concentration was 110 μg/l (64; 169 μg/l) in SHIP and 151 μg/l (97; 214 μg/l) in KORA, and the median TSH value was 0.81 mIU/l (0.56; 1.15 mIU/l) in SHIP and 1.22 mIU/l (0.84; 1.80 mIU/l) in KORA. The frequency of elevated TSH (TSH ≥ 2.12 mIU/l) was 4.3% in SHIP and 14.1% in KORA (P<0.001); the corresponding values for suppressed TSH (<0.25 mIU/l) were 3.5 and 1.7% (P<0.001). The proportion of ultrasonographic findings was 55.5% in SHIP and 68.0% in KORA. The frequency of serum TPO-Abs did not differ significantly between northeast and south Germany. CONCLUSIONS There were considerable regional disparities in the frequency of thyroid disorders within Germany. These differences can be explained not only by different regional histories of natural iodine deficiency but also by current differences in the iodine supply under an identical nationwide iodine fortification program.
Extinction risk under coloured environmental noise

Positively autocorrelated red environmental noise is characterized by a strong dependence of expected sample variance on sample length. This dependence has to be taken into account when assessing extinction risk under red and white uncorrelated environmental noise. To facilitate a comparison between red and white noise, their expected variances can be scaled to be equal, but only at a chosen time scale. We show with a simple one-dimensional population dynamics model that the different but equally reasonable choices of the time scale yield qualitatively different results on the dependence of extinction risk on the colour of environmental noise: extinction risk might increase as well as decrease when the temporal correlation of noise increases.
Construction and characterization of Streptococcus suis-Escherichia coli shuttle cloning vectors.

pSSU1, a native plasmid of Streptococcus suis DAT1, was used to construct pSET-series shuttle vectors. In addition to the replication function of pSSU1, these vectors contain the multiple cloning sites and lacZ' gene from pUC19, which means that X-gal screening can be used to select recombinants in Escherichia coli. pSET1, pSET2, and pSET3 carry cat, spc, and both of these genes, respectively, as selectable markers. These vectors could be introduced into S. suis, E. coli, Salmonella typhimurium, S. pneumoniae, and S. equi ssp. equi by electrotransformation. The recA gene was cloned from S. suis and sequenced, and this information was used in the construction of a recA mutant of S. suis. Transformation frequencies and/or plasmid stability of all pSET vectors tested were decreased in both S. suis and E. coli recA mutants compared with the parental strains. These results suggested that functional RecA protein improved the maintenance of pSET vectors in both S. suis and E. coli. Moreover, cloning of the functional S. suis recA gene into pSET2 and complementation analysis of the recA mutant were successful in S. suis but not in E. coli. These results showed that pSET vectors are useful tools for cloning and analyzing S. suis genes in S. suis strains directly.
Adolescent idiopathic scoliosis: Retrospective analysis of 235 surgically treated cases

Background: The surgical treatment of adolescent idiopathic scoliosis (AIS) has taken great strides in the last two decades. There have been no long term reported studies on AIS from India with documented long term followup. In this study we review a single surgical team’s series of 235 surgically treated cases of AIS with a follow-up from two to six years. Materials and Methods: Pre operative charts, radiographs and MRI scans for 235 patients were collected for this study. The patients were grouped into three groups where anterior correction and fusion (n=47), posterior correction and fusion (n=123) and combined anterior release and posterior instrumentation (n=65) was performed. Each group was divided into two subgroups based on the surgical approach and instrumentation strategy (all screw construct or hybrid construct) used. Patients were followed up for coronal and saggital plane corrections, apical vertebral translation (AVT), trunk balance and back pain. The percentage of correction was calculated in each group as well as sub groups. Results: The incidence of MRI detected intraspinal anomaly in this series is 5.9% with 3.4% of them requiring neurosurgical procedure along with scoliosis correction. Average coronal major curve correction was 66% in the all screw group and 58.5% in the hybrid group. The coronal plane correction was better when the all screw constructs were employed. Also, the AVT and trunk balance was better with the all screw constructs. The anterior corrections resulted in better correction of the AVT and trunk balance as compared to the posterior correction. There were eight (3.4%) complications in this series. The coronal and saggital plane correction paralleled the published international standards. Conclusion: The coronal plane correction was better when all screw constructs were employed. Use of all pedicle screw systems obviated the need for costoplasty in most cases. The increased incidence of intraspinal anomaly may warrant a routine pre operative MR imaging of all adolescent scoliosis needing surgical treatment.
Spin-Liquid Ground State of the S = 1/2 Kagome Heisenberg Antiferromagnet

Numerical calculations reveal that the true ground state of a frustrated two-dimensional system is a gapped spin liquid. We use the density matrix renormalization group to perform accurate calculations of the ground state of the nearest-neighbor quantum spin S = 1/2 Heisenberg antiferromagnet on the kagome lattice. We study this model on numerous long cylinders with circumferences up to 12 lattice spacings. Through a combination of very-low-energy and small finite-size effects, our results provide strong evidence that, for the infinite two-dimensional system, the ground state of this model is a fully gapped spin liquid.
Revenue diversification and total assets in commercial banks: Evidence from selected ASEAN countries

This study reveals the impact of total asset size upon revenue diversification in commercial banks in five of the countries in the Association of Southeast Asian Nations (ASEAN) countries – Indonesia, Malaysia, the Philippines, Thailand, and Vietnam – during the period between 2005 and 2015. By applying the General Moment Method (the GMM) to the unbalanced panel data, this research has determined the impact of the total asset size, as well as the impact of a number of other factors, such as non-performing loans, net interest margin rates, owner equity, business cycles, and the years of the financial crisis. The empirical results show the positive impacts of total asset size, non-performing loans, and the years of the financial crisis upon the levels of revenue diversification. However, other variables are negatively correlated with revenue diversification, such as net interest margin rates, owner equity, and business cycles.
Impact of silver nanoparticles on marine diatom Skeletonema costatum

When silver nanoparticles (AgNPs) are used commercially at a large scale, they infiltrate the environment at a rapid pace. However, the impact of large quantities of AgNPs on aquatic ecosystems is still largely unknown. In aquatic ecosystems, the phytoplanktons have a vital ecological function and, therefore, the potential impact of AgNPs on the microalgae community has elicited substantial concern. Therefore, in this study, the impacts of AgNPs on a marine diatom, the Skeletonema costatum, are investigated, with a focus on their photosynthesis and associated mechanisms. Exposure to AgNPs at a concentration of 0.5 mg l−1 significantly induces excess intracellular reactive oxygen species (ROS, 122%) and reduces 28% of their cell viability. More importantly, exposure to AgNPs reduces the algal chlorophyll‐a content. Scanning electron microscopy (SEM) was conducted, which revealed that AgNPs obstruct the light absorption of algae because they adhere to their surface. The maximum photochemical efficiency of photosystem II (Fv/Fm) demonstrates that exposure to AgNPs significantly inhibits the conversion of light energy into photosynthetic electron transport. Moreover, the genes of the photosystem II reaction center protein (D1) are significantly down‐regulated (P < 0.05) upon exposure to 5 mg l−1 AgNPs. These results suggest that the physical adhesion and effects of shading of AgNPs on algae might affect their light energy delivery system and damage the crucial protein function of PSII. The photosynthesis inhibition effect of AgNPs is largely different from Ag+. This study shows that AgNPs at higher concentrations might have serious consequences for the succession of the phytoplankton communities and aquatic ecosystem equilibrium. Copyright © 2016 John Wiley & Sons, Ltd.
Exploring the Relationship between Financial Distress, Financial Flexibility, and Firm Performance: Empirical Evidence from Pakistan Stock Exchange

Purpose– This main purpose of this paper is to empirically investigate the impact of corporate financial flexibility (FF) on financial distress and performance of firms listed on the Pakistan Stock Exchange (PSX). It enables to know how financial flexibility affects the firm financial strength, financial distress, and corporate performance. Design/methodology/approach –This study focuses on a firm level data of 192 non-financial firms covering the period 1992 - 2014. The fixed effect model logistic regression is applied by using unbalanced panel data to examine the impact of financial flexibility on financial distress, and performance of sample firms. Findings – The results reveal that financially flexible firms are less likely to face financial distress. As firms have more financial flexibility, the probability of financial distress decreases as well. It is also found that financially flexible firms are more likely to perform well than counterpart firms. By using the Altman z score as a measure of financial distress it is revealed that as the Altman z score increases, the chances of financial distress reduce as well. These findings also suggest the existence of pecking order in Pakistani firms; because firms rely on internal sources first, second go to external sources of financing. Practical implications – the findings of this study enable the corporate managers to avoid financial distress by obtaining and maintaining financial flexibility by keeping the leverage level lower than industry level. By attaining and maintaining financial flexibility, corporate managers can also raise the performance of the firm as well. It can also enable to make appropriate capital structure decision to finance managers of corporate firms. The creditors may provide the loan to sound firms who have no or least chances of financial distress. The lenders may also get benefit from it by requiring the interest rate as per risk of financial distress of the firm. Investors may avoid investing in firms having very little or no financial flexibility. JEL Classification– G33, L25 Keywords: Altman z score, financial flexibility, firm performance, return on asset, panel data, financial distress, modified z score.
Knowledge about glaucoma and barriers to follow-up care in a community glaucoma screening program.

OBJECTIVE To assess perceived barriers to glaucoma follow-up care, including the lack of glaucoma knowledge and the lack of health care access, among participants in a community glaucoma screening program. DESIGN Community survey. PARTICIPANTS Two hundred forty-three consecutive participants in a series of free glaucoma screenings between November 2002 and August 2003. METHODS The survey consisted of 20 questions designed to elicit knowledge of glaucoma and perception of potential barriers to follow-up care. Our aim was to find correlations between patient demographics and knowledge of glaucoma as well as perceived potential barriers to follow-up care. The data were analyzed using SPSS, v. 10.1. RESULTS The average age of the respondents was 70 years, and females predominated (66%). About half of the respondents knew of an eye doctor in their neighborhood, and 60% had had an eye examination in the past year. Two hundred twenty-two (91%) indicated they could get to an eye doctor if the screening examination indicated they needed a follow-up examination. Two hundred twenty (90.5%) had medical insurance. One hundred seventy-eight (73%) of the participants had heard of glaucoma; 71 (29%) identified an accurate definition of glaucoma. The level of education and the language spoken at home were correlated with both glaucoma awareness (p < 0.001; p < 0.001) and knowledge of an accurate definition of glaucoma (p < 0.001; p < 0.025). CONCLUSIONS In this population, a lack of adequate education about glaucoma may be more significantly associated with poor follow-up rates than a lack of access to care in those identified as glaucoma suspects.
Effect of sympathomimetic drugs in eliciting hypertensive responses to reserpine in the rat, after pretreatment with monoamineoxidase inhibitors

1 The effects of some rapidly metabolized sympathomimetic amines, such as β‐phenylethylamine and p‐tyramine, in eliciting hypertensive responses to reserpine in the anaesthetized rat, have been studied. 2 Retardation of metabolism, by pretreatment with the monoamineoxidase inhibitors iproniazid or phenelzine, causes β‐phenylethylamine (which in untreated rats has no effect) to induce hypertensive responses to reserpine. Tyramine and other hydroxy substituted phenylethylamines are much less active in this respect, probably because of relatively poor lipid solubility. 3 Hypertensive responses to reserpine are due to catecholamine release, which is believed to be from stores made accessible to indirectly acting sympathomimetic amines with high lipid solubility by an action of reserpine on cell membranes.