Miracle-dz/UniFAR-FTUs huggingface dataset:
Contribution of a slowly inactivating potassium current to the transition to firing of neostriatal spiny projection neurons.

1. Neostriatal spiny projection neurons display a prominent slowly depolarizing (ramp) potential and long latency to spike discharge in response to intracellular current pulses. The contribution of a slowly inactivating A-current (IAs) to this delayed excitation was investigated in a neostriatal slice preparation using current pulse protocols incorporating information based on the known voltage dependence, kinetics, and pharmacological properties of IAs. 2. Depolarizing intracellular current pulses evoked a slowly developing ramp potential that could last for seconds without reaching steady state and continued until either the pulse was terminated or spike threshold was reached. The slope of the ramp potential was dependent on the level of depolarization achieved by the membrane, and the apparent activation threshold for this ramp depolarization was approximately -65 mV. 3. Application of low concentrations of 4-aminopyridine (4-AP, 30-100 microM) or dendrotoxin (DTX, 30 nM), which are known to selectively block IAs, reduced both the slope of the ramp potential and the latency to first spike discharge. As has been described previously, blockade of inward Na+ and Ca2+ currents with tetrodotoxin (TTX, 1 microM) and cadmium (400 microM) also reduced the slope of the ramp depolarization. 4. A conditioning-test pulse protocol was used to examine the voltage dependence of inactivation of the ramp potential and long first spike latency. In the absence of a conditioning pulse, the test pulse evoked a slowly rising ramp potential and a spike with a long latency to discharge. A conditioning depolarization to approximately -60 mV decreased the slope of the ramp potential and the latency to first spike discharge evoked by the test pulse. A conditioning hyperpolarization to potentials below -100 mV, increased first spike latency. Application of a low concentration of 4-AP (100 microM) abolished the influence of prior membrane potential on the slope of the ramp depolarization and the latency to first spike discharge. 5. The kinetics of recovery from inactivation of the 4-AP-sensitive current were studied in the presence of TTX and cadmium by depolarizing cells to approximately -50 mV and then stepping to approximately -90 mV for increasing periods of time (0.5-5.0 s) before delivering a test pulse. The amplitude of the test pulse response decreased as a function of the hyperpolarizing step duration. When the test pulse response amplitudes were plotted against the hyperpolarizing step duration, the points reflected an exponential decay with an average time constant of 2.05 +/- 1.38 (SD) s. (ABSTRACT TRUNCATED AT 400 WORDS)
Fetal and infantile hemochromatosis

Hemochromatosis is the term coined by Von Recklinghausen in the late 19th century to describe the association of endocrine dysfunction (i.e., diabetes), cirrhosis, and abnormally bronze-colored skin. Observed mainly in adult men,1 it was later determined that abnormal iron deposition in diseased tissues was involved in its pathogenesis and that it was a familial condition. It was not until 1996 that the hemochromatosis gene HFE was discovered and that the homozygous mutation C282Y was determined to be the cause of the vast majority of cases of hereditary hemochromatosis.2 In the intervening decade, mutations of other genes whose products are involved in the regulation of the body’s iron status have been identified in patients expressing the phenotype of hemochromatosis, resulting in several disorders that now constitute “hereditary hemochromatosis. ” There are few patients with the classic hemochromatosis phenotype in whom no gene defect can be identified. Children—particularly young children—have never fit into the classic hemochromatosis phenotype. Clinical observation has shown that many years are required for patients with hereditary hemochromatosis to accrue the amount of excess iron that causes disease. Thus, decades before the discovery of HFE, rare patients with iron overload and organ failure or dysfunction beginning in childhood were classified differently. Children presenting signs of the disorder some years after birth were diagnosed with “juvenile hemochromatosis” while newborns simply had “neonatal hemochromatosis”. Mutations of newly described genes whose products are important in iron homeostasis have been determined to cause most cases of juvenile onset hereditary hemochromatosis. These findings have helped unravel the complexities of the system that regulates iron metabolism. Yet, the clinical and pathological findings in these children who rapidly accrue iron, perhaps even during intrauterine life, have shed some doubt as to whether neonatal hemochromatosis could possibly result from iron overload. This review covers the clinical gamut of hemochromatosis as it affects fetuses and infants. The distinct clinical and pathological findings of neonatal hemochromatosis are the main focus of discussion. The subjects of hereditary hemochromatosis and iron homeostasis have been ably reviewed by Pietrangelo1 and Fleming and Bacon.3 Herein, brief overviews of these subjects will set the stage for discussing emerging concepts related to the pathogenesis of neonatal hemochromatosis.
Preparation, release and physicochemical characterisation of ethyl butyrate and hexanal inclusion complexes with β- and γ-cyclodextrin

Abstract Complexes of ethyl butyrate and hexanal encapsulated by β-cyclodextrin (β-CD) and γ-cyclodextrin (γ-CD) were prepared by coprecipitation, and gas chromatography was used to quantity the flavour compounds in the complexes. The ethyl butyrate–γ-CD complex had the highest inclusion ratio (12.20%) followed by the ethyl butyrate-β-CD, hexanal-β-CD and hexanal-γ-CD complexes (11.29, 4.41 and 3.33%, respectively). Release experiments were performed under different relative humidities (RH 93, 75 and 52%) and temperatures (4 and 25 °C). The flavour release behaviours of the complexes were described by the Avrami equation. The rate of flavour release was enhanced with both increasing temperature and RH, although the effect of RH was stronger. Physicochemical characterisation using FT-IR, XRD, DSC and SEM analyses demonstrated that crystalline complexes were formed. Both β-CD and γ-CD were able to encapsulate ethyl butyrate and hexanal, and lower RH and temperature were more suitable for the storage of these complexes.
Effects of balance exercises on people with multiple sclerosis: a pilot study

Objective: To evaluate the effects of balance retraining in a sample of people with multiple sclerosis. Design: Randomized controlled trial. Setting: Rehabilitation unit. Subjects: A consecutive sample of 44 subjects was randomized into two experimental groups and one control group. The inclusion criteria were: ability to stand independently more than 30 seconds, ability to walk for 6 m. Interventions: Group 1 received balance rehabilitation to improve motor and sensory strategies. Group 2 received balance rehabilitation to improve motor strategy. Group 3 received treatments not specifically aimed at improving balance. Main outcome measure: Berg Balance Scale, Dynamic Gait Index and fall frequency were used to assess balance impairments. Dizziness Handicap Inventory and Activities-specific Balance Confidence were used to assess handicap and the level of balance confidence. Results: Frequency of falls post treatment was statistically different among groups (P = 0.0001); The Berg Balance Scale showed an overall statistically significant difference (P = 0.0008) among groups. Change pre—post scores were 6.7, 4.6 and 0.8 points for groups 1, 2 and 3. Dynamic Gait Index showed an overall near statistically significant difference among groups (P = 0.14), with change pre—post scores of 3.85, 1.6 and 1.75 points for groups 1, 2 and 3; after the exclusion of drop-outs a statistically significant difference was observed (P = 0.04). The self-administered tests (Activities-specific Balance Confidence and Dizziness Handicap Inventory) did not show clinically relevant improvements. Conclusions: Balance rehabilitation appeared to be a useful tool in reducing the fall rate and improving balance skills in subjects with multiple sclerosis. Exercises in different sensory contexts may have an impact in improving dynamic balance.
Sonological and Pathological Evaluation of Solitary Nodule Thyroid

Thyroid nodules are a common clinical problem. Epidemiologic studies have shown the prevalence of palpable thyroid nodules to be approximately 5% in women and 1% in men living in iodine sufficient parts of world (1) . Most of the thyroid nodules are benign and only less than 7% of thyroid nodules are malignant (2) . So it
Topographic Indications of Emerging Keratoconus in Teenage New Zealanders

Purpose: To screen a population of teenagers for emerging topographic signs of keratoconus (KC), with particular reference to ethnicity. Methods: Corneal topography, visual acuity, and an environmental risk factor analysis were included in a screening program designed to identify early indications of potential KC in teenagers. Two schools from central North Island, New Zealand, were invited to participate: one with predominantly Maori/Polynesian students and the other with students of mainly European descent. Results: A total of 198 Maori/Polynesian, 16.8 ± 1.05 years of age, and 243 European students, 16.2 ± 1.3 years of age, participated in the study. Corneal topography suggestive strongly of KC was evident in 3 Maori/Polynesian [1.2%; 95% confidence interval (CI): 0.3-4.3] and no European students (0%; 95% CI, 0%-1.5%). Anomalous corneal topography reminiscent of emerging KC in at least 1 eye were found in 19% (95% CI, 15.6%-22.9%). A significant difference (P = 0.0014) in the percentage of suspected KC was found between European students (12.9%; 95% CI, 9.2%-17.9%) and Maori/Polynesian students (26.9%; 95% CI, 21.0%-33.7%). In multivariate analyses, the significant independent predictors of probable or suspected KC included being Maori/Polynesian [odds ratio (OR) = 2.1; 95% CI, 1.25-3.54; P = 0.0052], increasing age (OR = 1.4; 95% CI, 1.10-1.80; P = 0.0067), and a history of hayfever (OR = 2.0; 95% CI, 1.16-3.59; P = 0.013). Topographical indicators that were associated significantly with suspected KC included central keratometry (P < 0.0001), astigmatism (P = 0.014), and inferior-superior asymmetry (P < 0.0001). Conclusions: Although only a longitudinal study will determine the proportion of true KC candidates, these data provide evidence of ethnicity-related differences in corneal topography in teenage New Zealanders.
Basic characteristics of uniaxial extension rheology: Comparing monodisperse and bidisperse polymer melts

We have carried out continuous and step uniaxial extension experiments on monodisperse and bidisperse styrene-butadiene random copolymers (SBR) to demonstrate that their nonlinear rheological behavior can be understood in terms of yielding through breakdown of the chain entanglement network and rubberlike rupture via non-Gaussian chain stretching leading to chain scission, respectively. In continuous extension, the sample with bidisperse molecular weight distribution showed greater resistance, due to double-networking, against the yielding-initiated failure. An introduction of 20% high molecular weight (106 g/mol) SBR to an SBR matrix (2.4 × 105 g/mol) could postpone the onset of nonuniform extension by as much as two Hencky strain units. In step extension, the bidisperse blends were also found to be more resistant to elastic breakup than the monodisperse matrix SBR. Rupture in both monodisperse and bidisperse SBR samples occurred when the finite chain extensibility was reached at sufficiently high rates.
Early prenatal diagnosis of iniencephaly associated with holoprosencephaly and cyclops

Introduction Neural tube defect (NTD) is one of the most common malformations, with an incidence of 1 – 5 per 1,000 pregnancies in Turkey and worldwide (Coskun et al. 2009; Stoll et al. 2011; Tuncbilek et al. 1999). Iniencephaly is one of the most severe and rare forms of NTD. We present a case of early prenatal diagnosis of a fetus with iniencephaly apertus and associated anomalies. We believe this report represents one of the earliest diagnosed cases of iniencephaly.
Clinical thromboembolic detterrent stockings application: Are thromboembolic detterrent stockings in practice matching manufacturers application guidelines

Background Thromboembolic detterrent (TED) stockings have been shown to be effective in the reduction of thromboembolic events in post operative patients. These manufactured stockings create graduated compression from ankle to calf. Aim To assess whether the manufacturers' recommendations for application were being met in a District general hospital setting and whether this achieved the desired gradient of compression. Methods We carried out pressure measurements on 100 legs in post-operative patients and recorded reasons for poorly fitting stockings. Pressure measurements were taken at standard positions around calf and ankle using a pre-calibrated subbandage pressure measuring device. Results About 20% of stockings were worn incorrectly by patients. Median pressure applied at the ankle was 13 mmHg (range, 6.5–18.5) compared to the manufacturers' intended compression of 18 mmHg. Only 14% of the stockings showed an acceptable gradation of reduced pressure between ankle and calf. About 23% of the stockings exerted a positive pressure at calf level compared to the ankle. Conclusion Most TED stockings do not produce a standardised Siegel profile pressure gradient decrease from ankle to calf. This may be due in part to fluid changes after surgery in combination with the large variation in size of lower limbs. Our District general hospital utilises three of the six sizes of TED stocking, and remeasurement was not taking place every 24 h as per guidance. This as the result show not only negates the benefit of TED stockings but may also exert harm in terms of venous thromboembolism risk. This finding adds further weight to the argument of whether TED stockings may not be having the desired prophylactic effect and may even be resulting in harm in select cases.
Comparison of biochemical composition of three aqua cultured fishes (Dicentrarchus labrax, Sparus aurata, Dentex dentex)

The moisture, ash, protein and fat content and amino acid, fatty acid and mineral compositions in the flesh of three commercially important fish species (Dicentrarchus labrax, Sparus aurata, Dentex dentex) from the aqua culture of Turkey were evaluated. Ranges of moisture, ash, protein and fat contents were 69.68–76.42%, 1.49–1.95%, 18.21–21.70% and 2.29–8.10%, respectively. Among them, those occurring in the highest proportions were aspartic acid (3238.91–5204.71 mg/100 g), glutamic acid (5256.60–5810.43 mg/100 g), arginine (198.07–414.91 mg/kg), phenylalanine (50.51–386.96 mg/100 g), leucine (375.84–574.94 mg/100 g) and lysine (3089.71–4711.98 mg/100 g). The fatty acid compositions of sea bass (D. labrax), sea bream (S. aurata) and common dentex (D. dentex) ranged from 28.01% to 32.41% saturated, from 25.88% to 28.62% monounsaturated, and from 24.75% to 27.42% polyunsaturated. The highest potassium, magnesium, phosphorus, iodine and selenium contents were found in these fishes.
Understanding the Relationships between Multi-dimensional Trust and E-loyalty in E-commerce

As online shopping is becoming more and more popular in recent years, many studies have been conducted to explore the key issues involved in online shopping such as trust problems and strategies to retain customers. This study will explore the nature of trust and its relationship to consumer loyalty in E-commerce. It examines the importance of trust to consumers’ loyalty behaviour and how consumer behaviour is affected by different kinds of trust. The study also identifies the antecedents of each kind of trust. A research model is developed with several hypotheses which are proposed for empirical examination. Finally a research plan is presented and implications are provided.
[Bone density and 25-OH vitamin D serum level in patients with systemic lupus erythematosus].

OBJECTIVE To clarify the influence of vitamin D metabolism on bone mineral density (BMD) or bone metabolism in patients with systemic lupus erythematosus (SLE). METHODS 57 consecutive patients in our department (mean age 33.9 years, 44 female, 13 male) were studied. BMD was measured with dual-X-ray absorptiometry at the lumbar spine and femoral neck. Biochemical investigation of bone metabolism included measurement of vitamin D metabolites, intact parathyroid hormone (PTH), serum osteocalcin und urinary pyridinoline-crosslink excretion. RESULTS 25 patients had 25-OH cholecalciferol serum values below the normal range after adjustment for seasonal changes; 9 patients were severely vitamin D depleted with 25-OH vitamin D serum values below 5 ng/ml. Low 25-OH-vitamin D was significantly associated with high disease activity. Mean 1.25 (OH)2-vitamin D, PTH, osteocalcin and crosslink excretion were in the normal range. Thirty-six patients had normal BMD; 5 patients had osteoporosis according to WHO diagnosis criteria. No correlation of biochemical parameters of bone metabolism with BMD was found. CONCLUSION Severe vitamin D depletion was common in this group of patients with SLE even after adjustment for seasonal variations, especially in patients with high disease activity. Therefore, D-hypovitaminosis should be included in the differential diagnosis in patients with SLE presenting with low bone mass.
Oxygen Saturation in Immature Babies: Revisited with Updated Recommendations

The present data therefore clearly indicate that a low saturation is beneficial with respect to minimizing both eye and lung problems in ELBW infants. Clinical recommendations, such as the recent European guidelines for management of respiratory distress syndrome, therefore recommend SpO 2 targets between 85 and 93%. The level of evidence for these recommendations was, however, considered low, based on case series, case reports or expert opinion [5] .
Failure of Hairpin-Ended and Nicked DNA To Activate DNA-Dependent Protein Kinase: Implications for V(D)J Recombination

ABSTRACT V(D)J recombination is initiated by a coordinated cleavage reaction that nicks DNA at two sites and then forms a hairpin coding end and blunt signal end at each site. Following cleavage, the DNA ends are joined by a process that is incompletely understood but nevertheless depends on DNA-dependent protein kinase (DNA-PK), which consists of Ku and a 460-kDa catalytic subunit (DNA-PKCS or p460). Ku directs DNA-PKCS to DNA ends to efficiently activate the kinase. In vivo, the mouse SCID mutation in DNA-PKCSdisrupts joining of the hairpin coding ends but spares joining of the open signal ends. To better understand the mechanism of V(D)J recombination, we measured the activation of DNA-PK by the three DNA structures formed during the cleavage reaction: open ends, DNA nicks, and hairpin ends. Although open DNA ends strongly activated DNA-PK, nicked DNA substrates and hairpin-ended DNA did not. Therefore, even though efficient processing of hairpin coding ends requires DNA-PKCS, this may occur by activation of the kinase bound to the cogenerated open signal end rather than to the hairpin end itself.
Analysis of different converters used in wind energy conversion system

This paper studies performances of wind energy conversion system (WECS) using Permanent Magnet Synchronous Generator (PMSG). The big interest and requirements to high efficiency imposes the wind turbine to operate in variable speed mode, which inevitably requires power electronic converters to maximize the extracted power from the wind as well as decoupling generator dynamics from the grid or load. The aim of this paper is to present an analysis of the suitable topology among the most popular rectifier systems for the generator-side converter those are: the passive diode bridge rectifier and the active six-switch two level rectifier, another rectifier system considered in this study include combinations of a diode bridge rectifier and DC-DC converter, the half controlled rectifier topology is also discussed. This work provides a comparison study between performances of different rectifier systems to examine their performances in (WECSs) starting by modeling of the used converters in the rectifier system, then a comparison is made between the association of (PMSG-diode rectifier) and (PMSG- diode rectifier with DC-DC buck converter), then with fully controlled rectifier, in order to discover their influences on the performances of the PMSG and the wind turbine system. Simulation results using Matlab Similink are presented to support the conclusion.
Expert opinion on pharmacotherapy of kidney disease in HIV-infected patients

Introduction: Human immunodeficiency virus (HIV) infection is associated with the development of a wide spectrum of kidney diseases. HIV-associated nephropathy (HIVAN) is the most common cause of chronic kidney disease (CKD) in HIV-infected individuals and predominantly affects patients of African ancestry. HIVAN is a leading cause of end-stage renal disease (ESRD) among African–Americans. Areas covered: An overview of the spectrum of kidney disease in patients with HIV is given. Current pharmacologic interventions to treat kidney disease in HIV are discussed. This review will enhance knowledge regarding the most common causes of kidney disease in HIV-infected patients. An understanding of the principles related to pharmacotherapy in HIV-infected patients with kidney disease will also be gained. Expert opinion: Kidney disease is an important cause of morbidity and mortality in HIV-infected patients. The most common cause of chronic kidney disease in this population is HIV-associated nephropathy, which is caused by viral infection of the renal epithelium. Several medications that are commonly used in HIV-infected patients can have adverse effects on the kidneys and the doses of many antiretroviral medications need to be adjusted in patients with impaired renal function.
Specific Association of a Set of Molecular Chaperones Including HSP90 and Cdc37 with MOK, a Member of the Mitogen-activated Protein Kinase Superfamily*

We have recently identified and cloned a novel member of mitogen-activated protein kinase superfamily protein, MOK (Miyata, Y., Akashi, M., and Nishida, E. (1999) Genes Cells 4, 299–309). To address its regulatory mechanisms, we searched for cellular proteins that specifically associate with MOK by co-immunoprecipitation experiments. Several cellular proteins including a major 90-kDa molecular chaperone HSP90 were found associated with MOK. Treatment of cells with geldanamycin, an HSP90-specific inhibitor, rapidly decreased the protein level of MOK, and the decrease was attributed to enhanced degradation of MOK through proteasome-dependent pathways. Our data suggest that the association with HSP90 may regulate intracellular protein stability and solubility of MOK. Experiments with a series of deletion mutants of MOK indicated that the region encompassing the protein kinase catalytic subdomains I–IV is required for HSP90 binding. Closely related protein kinases (male germ cell-associated kinase and male germ cell-associated kinase-related kinase) were also found to associate with HSP90, whereas conventional mitogen-activated protein kinases (extracellular signal-regulated kinase, p38, and c-Jun N-terminal kinase/stress-activated protein kinase) were not associated with HSP90. In addition, we found that other molecular chaperones including Cdc37, HSC70, HSP70, and HSP60 but not GRP94, FKBP52, or Hop were detected specifically in the MOK-HSP90 immunocomplexes. These results taken together suggest a role of a specific set of molecular chaperones in the stability of signal-transducing protein kinases.