Miracle-dz/UniFAR-FTUs huggingface dataset:
Glycosaminoglycan Storage Disorders: A Review

Impaired degradation of glycosaminoglycans (GAGs) with consequent intralysosomal accumulation of undegraded products causes a group of lysosomal storage disorders known as mucopolysaccharidoses (MPSs). Characteristically, MPSs are recognized by increased excretion in urine of partially degraded GAGs which ultimately result in progressive cell, tissue, and organ dysfunction. There are eleven different enzymes involved in the stepwise degradation of GAGs. Deficiencies in each of those enzymes result in seven different MPSs, all sharing a series of clinical features, though in variable degrees. Usually MPS are characterized by a chronic and progressive course, with different degrees of severity. Typical symptoms include organomegaly, dysostosis multiplex, and coarse facies. Central nervous system, hearing, vision, and cardiovascular function may also be affected. Here, we provide an overview of the molecular basis, enzymatic defects, clinical manifestations, and diagnosis of each MPS, focusing also on the available animal models and describing potential perspectives of therapy for each one.
Origins of Cortical Interneuron Subtypes

Cerebral cortical functions are conducted by two general classes of neurons: glutamatergic projection neurons and GABAergic interneurons. Distinct interneuron subtypes serve distinct roles in modulating cortical activity and can be differentially affected in cortical diseases, but little is known about the mechanisms for generating their diversity. Recent evidence suggests that many cortical interneurons originate within the subcortical telencephalon and then migrate tangentially into the overlying cortex. To test the hypothesis that distinct interneuron subtypes are derived from distinct telencephalic subdivisions, we have used an in vitro assay to assess the developmental potential of subregions of the telencephalic proliferative zone (PZ) to give rise to neurochemically defined interneuron subgroups. PZ cells from GFP+ donor mouse embryos were transplanted onto neonatal cortical feeder cells and assessed for their ability to generate specific interneuron subtypes. Our results suggest that the parvalbumin- and the somatostatin-expressing interneuron subgroups originate primarily within the medial ganglionic eminence (MGE) of the subcortical telencephalon, whereas the calretinin-expressing interneurons appear to derive mainly from the caudal ganglionic eminence (CGE). These results are supported by findings from primary cultures of cortex from Nkx2.1 mutants, in which normal MGE fails to form but in which the CGE is less affected. In these cultures, parvalbumin- and somatostatin-expressing cells are absent, although calretinin-expressing interneurons are present. Interestingly, calretinin-expressing bipolar interneurons were nearly absent from cortical cultures of Dlx1/2 mutants. By establishing spatial differences in the origins of interneuron subtypes, these studies lay the groundwork for elucidating the molecular bases for their distinct differentiation pathways.
Health-related behavior and quality of life among the elderly: a population-based study.

OBJECTIVE To assess the association between health-related behaviors and quality of life among the elderly. METHODS A population-based cross-sectional study was carried out including 1,958 elderly living in four areas in the state of São Paulo, southeastern Brazil, 2001/2002. Quality of life was assessed using the Medical Outcomes Study SF-36-Item Short Form Health Survey instrument. This instrument's eight subscales and two components were the dependent variables. Independent variables were physical activity, weekly frequency of alcohol consumption and smoking. Multiple linear regression models were used to control for the effect of gender, age, schooling, work, area of residence and number of chronic conditions. RESULTS Physical activity was positively associated with the eight SF-36 subscales. The stronger associations were found for role-physical (β=11.9), physical functioning (β=11.3) and physical component. Elderly individuals who consumed alcohol at least once a week showed a better quality of life than those did not consume alcohol. Compared to non-smokers, smokers had a poorer quality of life for the mental component (β=-2.4). CONCLUSIONS The study results showed that physical activity, moderate alcohol consumption and no smoking are positively associated with a better quality of life in the elderly.
Homoclinic Points of Algebraic Z D -actions

Let α be an action of Z d by continuous automorphisms of a compact abelian group X. A point x in X is called homoclinic for α if α n x → 0X as n → ∞. We study the set ∆α(X) of homoclinic points for α, which is a subgroup of X. If α is expansive then ∆α(X) is at most countable. Our main results are that if α is expansive, then (1) ∆α(x) is nontrivial if and only if α has positive entropy and (2) ∆α(X) is nontrivial and dense in X if and only if α has completely positive entropy. In many important cases ∆α(X) is generated by a fundamental homoclinic point which can be computed explicitly using Fourier analysis. Homoclinic points for expansive actions must decay to zero exponentially fast, and we use this to establish strong specification properties for such actions. This provides an extensive class of examples of Z d-actions to which Ruelle's thermodynamic formalism applies. The paper concludes with a series of examples which highlight the crucial role of expansiveness in our main results.
The Immunoexpression of Heparanase 2 in Normal Epithelium, Intraepithelial, and Invasive Squamous Neoplasia of the Cervix

Objective Heparanase 2 (HPSE2) is expressed in various tissues, including the brain, intestine, prostate, breast, and endometrium. The aim of this study was to investigate the role of HPSE2 in cervical carcinogenesis, which has not been clarified to date. Materials and Methods The immunoexpression of HPSE2 in normal and neoplastic cervical squamous epithelia was determined using a semiquantitative (SQ) method and an index of expression (IE) method, using Image Lab Software. A total of 230 cervical tissue samples were analyzed and segregated into the following diagnostic groups: normal (27.4%), cervical intraepithelial neoplasia 1 (CIN 1, 15.2%), CIN 2 (16.5%), CIN 3 (15.2%), and invasive neoplasia (25.7%). The mean HPSE2 expression in the normal group was significantly lower than that of the other groups individually or combined (p < .001, for all combinations). The immunoexpression via the SQ method was significantly greater in the CIN 3 group compared with that in the CIN 1 group (p = .02). The mean immunoexpression of the high-grade squamous intraepithelial lesion groups was significantly greater than those of the normal and low-grade squamous intraepithelial lesion groups (p < .001) and lower compared with that of the invasive neoplasia group (p < .001). There were no statistically significant differences in the immunoexpression of HPSE2 among the different clinical states within the invasive neoplasia group. Conclusions The SQ method produced a greater sensitivity and specificity than did the index of expression method. There was a progressive increase in the mean HPSE2 immunoexpression according to the severity of the cervical lesion from the low-grade squamous intraepithelial lesion group to the invasive neoplasm group, whereas the normal group displayed the lowest level of expression. This is a novel study concerning HPSE2 in the cervix and cervical cancer carcinogenesis.
Determinants of mammography screening behavior in Iranian women: A population-based study

Background: Breast cancer remains a substantial health concern in Iran due to delay and late stage at diagnosis and treatment. Despite the potential benefits of mammography screening for early detection of breast cancer, the performance of this screening among Iranian women is low. For planning appropriate intervention, this study was carried out to identify mammography rates and explore determinants of mammography screening behavior in females of Isfahan, Iran. Materials and Methods: In this population-based study, 384 women of 40 years and older were interviewed by telephone. The Farsi version of Champion's Health Belief Model scale (CHBMS) was used to examine factors associated with mammography screening. The obtained data were analyzed by SPSS (version 16.0) using statistical Chi-square, Fisher Exact test, t-test and multiple logistic regression model to identify the importance rate of socio-demographic and Health Belief Model (HBM) variables to predict mammography screening behavior. In all of tests, the level of significant was considered a = 0.05. Results: Mean age ± SD of women was 52.24 ± 8.2 years. Of the 384 participants, 44.3% reported at least one mammogram in their lifetime. Logistic regression analysis indicated that women were more likely to have mammography if they heard/read about breast cancer (OR = 4.17, 95% CI 2.09, 8.34), menopause in lower age (OR = 0.2, 95% CI 0.87, 0.99) and history of breast problem (OR = 0.9, 95% CI 0.12, 0.32). Also, women who perceived more benefits of mammography (OR = 1.84, 95% CI 1.63, 2.09), fewer barriers of mammography (OR = 0.91, 95% CI 0.86, 0.96) and had more motivation for health (OR = 0.94, 95% CI 0.89, 1) were more likely to have mammography. Conclusion: The findings indicated that the rate of mammography screening among women in Isfahan province is low and highlights the need for developing a comprehensive national breast cancer control program, which should be considered as the first priority for healthcare providers. Also, identification of these factors can help to design an appropriate educational intervention that focuses on benefits of mammography screening, decreasing changeable barriers, improving access to mammography, increasing health motivation, promoting perceived self-efficacy and mammography adherence.
Preclinical Studies of a Modified Vaccinia Virus Ankara-Based HIV Candidate Vaccine: Antigen Presentation and Antiviral Effect

ABSTRACT Poxvirus-based human immunodeficiency virus (HIV) vaccine candidates are currently under evaluation in preclinical and clinical trials. Modified vaccinia virus Ankara (MVA) vectors have excellent safety and immunogenicity records, but their behavior in human cell cultures remains only partly characterized. We studied here various virological and immunological aspects of the interactions of MVA-HIV, a vaccine candidate developed by the French National Agency for AIDS Research (ANRS), with primary human cells. We report that MVA-HIV infects and drives Gag expression in primary macrophages, dendritic cells (DCs), and epithelial and muscle cells. MVA-HIV-infected DCs matured, efficiently presented Gag, Pol, and Nef antigens, and activated HIV-specific cytotoxic T lymphocytes (CTLs). As expected with this type of vector, infection was cytopathic and led to DC apoptosis. Coculture of MVA-HIV-infected epithelial cells or myotubes with DCs promoted efficient Gag antigen major histocompatibility complex class I (MHC-I) cross-presentation without inducing direct infection and death of DCs. Antigen-presenting cells (APCs) infected with MVA-HIV also activated HIV-specific CD4+ T cells. Moreover, exposure of DCs to MVA-HIV or to MVA-HIV-infected myotubes induced type I interferon (IFN) production and inhibited subsequent HIV replication and transfer to lymphocytes. Altogether, these results show that MVA-HIV promotes efficient MHC-I and MHC-II presentation of HIV antigens by APCs without facilitating HIV replication. Deciphering the immune responses to MVA in culture experiments will help in the design of innovative vaccine strategies.
Facial Palsy in Children: Emergency Department Management and Outcome

Objective: To describe the characteristics of children who present to an emergency department (ED) with facial palsy and determine the association of outcome with etiology, degree of initial paralysis, and ED management. Methods: This was a retrospective cohort study of children who presented to an ED with facial nerve paralysis (FNP). Results: There were 85 patients with a mean age of 8.0 (SD, 6.1) years; 60% (n = 51) of the patients were male, and 65.9% (n = 56) were admitted to the hospital. Bell palsy (50.6%) was the most common etiology followed by infectious (22.4%), traumatic (16.5%), congenital (7.1%), and neoplastic etiologies (3.5%). Patients with Bell palsy had shorter recovery times (P = 0.049), and traumatic cases required a longer time for recovery (P = 0.016). Acute otitis media (AOM)-related pediatric FNP had shorter recovery times than non-AOM-related cases (P = 0.005) in infectious group. Patients given steroid therapy did not have a shorter recovery time (P = 0.237) or a better recovery (P = 0.269). There was no difference in recovery rate of pediatric patients with Bell palsy between hospitalization or not (P = 0.952). Conclusion: Bell palsy, infection, and trauma were most common etiologies of pediatric FNP. Recovery times were shorter in pediatric patients with Bell palsy and AOM-related FNP, whereas recovery took longer in traumatic cases. Steroid therapy did not seem beneficial for pediatric FNP. Hospitalization is not indicated for pediatric patients with Bell palsy.
PHYTOREMEDIATION OF INORGANICS: REALISM AND SYNERGIES

There are very few practical demonstrations of the phytoextraction of metals and metalloids from soils and sediments beyond small-scale and short-term trials. The two approaches used have been based on using 1) hyperaccumulator species, such as Thlaspi caerulescens (Pb, Zn, Cd, Ni), Alyssum spp. (Ni, Co), and Pteris vittata (As) or 2) fast-growing plants, such as Salix and Populus spp. that accumulate above-average concentrations of only a smaller number of the more mobile trace elements (Cd, Zn, B). Until we have advanced much more along the pathway of genetic isolation and transfer of hyperaccumulator traits into productive plants, there is a high risk in marketing either approach as a technology or stand-alone solution to clean up contaminated land. There are particular uncertainties over the longer-term effectiveness of phytoextraction and associated environmental issues. Marginally contaminated agricultural soils provide the most likely land use where phytoextraction can be used as a polishing technology. An alternative and more useful practical approach in many situations currently would be to give more attention to crops selected for phytoexclusion: selecting crops that do not translocate high concentrations of metals to edible parts. Soils of brownfield, urban, and industrial areas provide a large-scale opportunity to use phytoremediation, but the focus here should be on the more realistic possibilities of risk-managed phytostabilization and monitored natural attenuation. We argue that the wider practical applications of phytoremediation are too often overlooked. There is huge scope for cross-cutting other environmental agenda, with synergies that involve the recovery and provision of services from degraded landscapes and contaminated soils. An additional focus on biomass energy, improved biodiversity, watershed management, soil protection, carbon sequestration, and improved soil health is required for the justification and advancement of phytotechnologies.
Electrodeposition and characterization of hydroxyapatite coatings doped by Sr2+, Mg2+, Na+ and F− on 316L stainless steel

Hydroxyapatite (HAp) co-doped by magnesium, strontium, sodium and fluorine was deposited on the 316L stainless steel (316LSS) substrates by electrodeposition method in electrolyte containing: , , , NaF, and . The influences of scanning potential ranges and temperature on MgSrFNaHAp coatings were researched. The obtained coatings under the optimum condition of scanning potential range from 0 to −1.7 V/SCE, at 50 °C have single phase crystals of HAp, cylinder shape and the thickness of 8.9 µm. The tests of 316LSS, NaHAp/316LSS and MgSrFNaHAp/316LSS materials in physiological saline and simulated body fluid (SBF) solutions were carried out. The results showed that the dissolution of MgSrFNaHAp coatings was lower than that of NaHAp ones and open circuit potential (OCP) of MgSrFNaHAp/316LSS was higher than those of NaHAp/316LSS and 316LSS at any immersion time. After 21 immersion days, the impedance modulus of MgSrFNaHAp/316LSS reached , which was 4.2 and 1.7 times higher than those of 316LSS and NaHAp/316LSS, respectively. The corrosion current density of MgSrFNaHAp/316LSS decreased about 2.3 and 7.8 times in comparison with that of NaHAp/316LSS and 316LSS, respectively. These results indicated that MgSrFNaHAp coatings can protect for the 316LSS substrates better than NaHAp coatings.
Antecedents of Project Implementation Success in crowdfunding

Crowdfunding is emerging as a booming financing channel for entrepreneurs to raise money for their projects. However, crowdfunding project implementation has been a major challenge which does not gain enough attention in the current literature. This paper developed a research model to investigate the antecedents of crowdfunding project success measured in three dimensions, i.e., award (product) delivery timeliness, the extent to which the award meets the specifications (meeting specifications), and sponsor overall satisfaction. We conducted a survey in Demohour, one of the famous crowdfunding platforms in China, to test the proposed model. The findings suggested that compared with delivery timeliness, meeting specifications is of more importance to increase overall satisfaction. The results also revealed that crowdfunding project difficulty significantly reduces the implementation success. In addition, team experience plays an important role in improving project success. Project planning is also a critical predictor for delivery timeliness. Both of theoretical research and crowdfunding industrial practice can draw some enlightenment from this study.
Initial-Condition Dependence and Initial-Condition Uncertainty in Climate Science

This article examines initial-condition dependence and initial-condition uncertainty for climate projections and predictions. The first contribution is to provide a clear conceptual characterization of predictions and projections. Concerning initial-condition dependence, projections are often described as experiments that do not depend on initial conditions. Although prominent, this claim has not been scrutinized much and can be interpreted differently. If interpreted as the claim that projections are not based on estimates of the actual initial conditions of the world or that what makes projections true are conditions in the world, this claim is true. However, it can also be interpreted as the claim that simulations used to obtain projections are independent of initial-condition ensembles. This article argues that evidence does not support this claim. Concerning initial-condition uncertainty, three kinds of initial-condition uncertainty are identified (two have received little attention from philosophers so far). The first (the one usually discussed) is the uncertainty associated with the spread of the ensemble simulations. The second arises because the theoretical initial ensemble cannot be used in calculations and has to be approximated by finitely many initial states. The third uncertainty arises because it is unclear how long the model should be run to obtain potential initial conditions at pre-industrial times. Overall, the discussion shows that initial-condition dependence and uncertainty in climate science are more complex and important issues than usually acknowledged. 1. Introduction2. Projections and Predictions 2.1. Predictions2.2. Projections3. Initial-Condition Dependence 3.1. Projections 3.1. 1. Dynamical conditions justifying independence of initial conditions? 3.1. 2. What projections can and cannot provide3.2. Predictions4. Initial-Condition Uncertainty 4.1. Projections4.2. Predictions5. Conclusion Appendix Introduction Projections and Predictions 2.1. Predictions2.2. Projections Predictions Projections Initial-Condition Dependence 3.1. Projections 3.1. 1. Dynamical conditions justifying independence of initial conditions? 3.1. 2. What projections can and cannot provide3.2. Predictions Projections 3.1. 1. Dynamical conditions justifying independence of initial conditions? 3.1. 2. What projections can and cannot provide Dynamical conditions justifying independence of initial conditions? What projections can and cannot provide Predictions Initial-Condition Uncertainty 4.1. Projections4.2. Predictions Projections Predictions Conclusion Appendix
Computational Fluid Dynamics (CFD) Simulation of Microfluidic Focusing in a Low-Cost Flow Cytometer

approved:_____________________________________________________ Nordica MacCarty Inertial microfluidics refers to the manipulation of the location and velocity of particles suspended in microscale fluid flow without the introduction of outside forces. It has promising applications in flow cytometry and other biomedical technologies because of the potential for lowering cost and complexity relative to current particle focusing methods. Utilizing inertial microfluidics requires accurately modeling the effects of inertial forces acting on particles. This paper explores a method of predicting the trajectory of microscale particles using the Discrete Phase Model of the ANSYS Fluent CFD software. A model was developed for the effects of inertial lift on solid particles in Poiseuille flow. This method was observed to have good agreement with empirical results when modeling the behavior of neutrally buoyant spherical particles experiencing non-negligible laminar flow in straight circular channels. The particles were observed to focus to an equilibrium annulus of about 0.6 the diameter of the channel. The speed with which they focused was observed to vary proportionally with particle diameter and Reynolds number, corresponding with empirical results.
On the relation between the Mason number and the durability of MR fluids

The durability of magnetorheological (MR) fluid is a fundamental aspect of any MR device, as MR fluid is submitted to shear stresses that cause its degradation. While various studies have identified MR fluid degradation behaviors and proposed improvements, there exists no generalized failure theory to help understand the in-use degradation of MR fluids. In this regard, this study suggests that a relation exists between the Mason number and the maximum achievable lifetime dissipated energy (LDE) of MR fluids. To validate this hypothesis, custom (perfluoropolyether) and commercial (hydrocarbon) MR fluids are aged in various Mason number conditions using a clutch-type durability device. Experimental results show that under a critical Mason number of ∼1, LDE of the MR fluids can be related to the Mason Number using an analogy to metal fatigue failure. Above this critical Mason number, the fundamental degradation mechanism appears to change from a dominant mechanical wear of the particles (i.e. particle-limited) to another mechanism (i.e. fluid-limited) which is not yet fully understood. Since MR generally operate well below the critical Mason number of ∼1, the relation between the Mason number and the particle-limited regime durability of MR fluids can be very useful to design novel MR fluids or MR devices, as it provides a way to estimate the impact of various design strategies on the total expected life of the device.