Miracle-dz/UniFAR-FTUs huggingface dataset:
Mutations in the gene encoding p62 in Japanese patients with amyotrophic lateral sclerosis

Objective: The purpose of this study was to find mutations in the SQSTM1 gene encoding p62 in Japanese patients with amyotrophic lateral sclerosis (ALS), since this gene has been recently identified as a causative gene for familial and sporadic ALS in the United States. Methods: We sequenced this gene in 61 Japanese patients with sporadic and familial ALS. To our knowledge, we describe for the first time the clinical information of such mutation-positive patients. Results: We found novel mutations, p.Ala53Thr and p.Pro439Leu, in 2 patients with sporadic ALS. The clinical picture of the mutation-positive patients was that of typical ALS with varied upper motor neuron signs. Although this gene is causative for another disease, Paget disease of bone (PDB), none of our patients showed evidence of concomitant PDB. Conclusion: The presence of mutations in this racial population suggests worldwide, common involvement of the SQSTM1 gene in ALS.
Salicylic acid alters antioxidant and phenolics metabolism in Catharanthus roseus grown under salinity stress.

BACKGROUND Salicylic acid (SA) acts as a potential non-enzymatic antioxidant and a plant growth regulator, which plays a major role in regulating various plant physiological mechanisms. The effects of salicylic acid (SA; 0.05 mM) on physiological parameters, antioxidative capacity and phenolic metabolism, lignin, alkaloid accumulation in salt stressed Catharanthus roseus were investigated. MATERIALS AND METHODS Catharanthus roseus seeds were grown for two months in a glass house at 27-30°C in sunlight, and then divided into four different groups and transplanted with each group with the following solutions for one month: group I (non-saline control), group II, 100 mM NaCl, group III, 0.05 mM SA, group IV, 100 mM NaCl+0.05 mM SA and to determine the physiological parameters (DW, FW, WC), chlorophyll contents, carotenoid contents, lipid peroxidation, phenolics, lignin, alkaloid and enzymatic assays in each leaf pairs and roots. RESULTS SA exhibited growth-promoting property, which correlated with the increase of dry weight, water content, photosynthetic pigments and soluble proteins. SA has additive effect on the significant increase in phenylalanine ammonia-lyase (PAL) activity, which is followed by an increase in total soluble phenolics and lignin contents in all leaf pairs and root of C. roseus. SA enhances malondialdehyde content in all leaf pairs and root. The antioxidant enzymes (catalase, glutathione reductase, glutathione-S-tranferase, superoxide dismutase, peroxidase) as well as alkaloid accumulation increased in all treatments over that of non-saline control but the magnitude of increase was found more in root. Further, the magnitude of increase of alkaloid accumulation was significantly higher in 100 mM NaCl, but highly significant was found in presence of 0.05 mM SA and intermediate in presence of both 0.05 mM SA+100 mM NaCl. CONCLUSION We concluded that applied SA to salt stress, antioxidant and phenolic metabolism, and alkaloid accumulation were significantly altered and the extent of alteration varied between the SA and salt stress.
The Protective Effects of Magnesium on Liver Tissue Alteration Induced by Bile Duct Ligation in Rat

Magnesium is the fourth most abundant cation in the body and the second most abundant cation in intracellular fluid. Magnesium plays an important role in numerous biological functions. In the present study we investigated the effect of magnesium sulfate (MgSO4) on histological abnormalities induced by bile duct ligation in male Wistar rats. The effects of 28-day oral administration of MgSO4 (at doses of 0.01, 0.05, 0.1, and 0.2 g/kg bw) on the levels of liver parameters, were evaluated in normal and BDL-induced cholestatic rats. Histopathological studies confirmed the protective effects of MgSO4 on cholestasis-induced hepatic injury in rats. the results of this study suggest that MgSO4 treatment may be beneficial in cholestasis-induced hepatotoxicity.
Humoral Immunity Links Candida albicans Infection and Celiac Disease

Objective The protein Hwp1, expressed on the pathogenic phase of Candida albicans, presents sequence analogy with the gluten protein gliadin and is also a substrate for transglutaminase. This had led to the suggestion that C. albicans infection (CI) may be a triggering factor for Celiac disease (CeD) onset. We investigated cross-immune reactivity between CeD and CI. Methods Serum IgG levels against recombinant Hwp1 and serological markers of CeD were measured in 87 CeD patients, 41 CI patients, and 98 healthy controls (HC). IgA and IgG were also measured in 20 individuals from each of these groups using microchips sensitized with 38 peptides designed from the N-terminal of Hwp1. Results CI and CeD patients had higher levels of anti-Hwp1 (p=0.0005 and p=0.004) and anti-gliadin (p=0.002 and p=0.0009) antibodies than HC but there was no significant difference between CeD and CI patients. CeD and CI patients had higher levels of anti-transglutaminase IgA than HC (p=0.0001 and p=0.0039). During CI, the increase in anti-Hwp1 paralleled the increase in anti-gliadin antibodies. Microchip analysis showed that CeD patients were more reactive against some Hwp1 peptides than CI patients, and that some deamidated peptides were more reactive than their native analogs. Binding of IgG from CeD patients to Hwp1 peptides was inhibited by γIII gliadin peptides. Conclusions Humoral cross-reactivity between Hwp1 and gliadin was observed during CeD and CI. Increased reactivity to Hwp1 deamidated peptide suggests that transglutaminase is involved in this interplay. These results support the hypothesis that CI may trigger CeD onset in genetically-susceptible individuals.
Inflammation, Vasospasm, and Brain Injury after Subarachnoid Hemorrhage

Subarachnoid hemorrhage (SAH) can lead to devastating neurological outcomes, and there are few pharmacologic treatments available for treating this condition. Both animal and human studies provide evidence of inflammation being a driving force behind the pathology of SAH, leading to both direct brain injury and vasospasm, which in turn leads to ischemic brain injury. Several inflammatory mediators that are elevated after SAH have been studied in detail. While there is promising data indicating that blocking these factors might benefit patients after SAH, there has been little success in clinical trials. One of the key factors that complicates clinical trials of SAH is the variability of the initial injury and subsequent inflammatory response. It is likely that both genetic and environmental factors contribute to the variability of patients' post-SAH inflammatory response and that this confounds trials of anti-inflammatory therapies. Additionally, systemic inflammation from other conditions that affect patients with SAH could contribute to brain injury and vasospasm after SAH. Continuing work on biomarkers of inflammation after SAH may lead to development of patient-specific anti-inflammatory therapies to improve outcome after SAH.
Suppression of Rous sarcoma virus-induced tumor formation by preinfection with viruses encoding src protein with novel N termini

Two recovered avian sarcoma viruses (rASVs), rASV157 and rASV1702, encode src products which contain novel, nonmyristoylated N-terminal amino acids. These viruses transform chicken embryo fibroblasts and cause tumors in chicks. However, the tumors rASVs induce are small and regress within 2 weeks. To determine whether this regression results from weak tumorigenicity or from the active immunity of the host, we injected 1-week-old chicks with rASV and several days later injected the chicks with challenge virus of a different subgroup. Of the rASV1702-preinfected chicks challenged 5 days later with Rous sarcoma virus (RSV), 40% showed no subsequent tumor formation and 60% formed tumors which regressed within 1 week. The potency of this protective effect depended on the dosage of preinfection virus used and increased as the interval between preinfection and challenge infection was lengthened (when the interval was 9 days, none of the challenged chicks formed tumors). rASV157-preinfected chicks challenged with RSV after 9 days showed only partial protection: 42% formed tumors which regressed, whereas 58% formed tumors which continued to grow. Challenging rASV-preinfected chicks with Fujinami sarcoma virus or a RSV vector encoding the v-fps oncogene or polyomavirus middle T resulted in no suppression of tumor formation. Preinfection with src mutants or a RSV vector encoding polyomavirus middle T antigen, both of which induce slow-growing tumors, failed to elicit the protective effect. Finally, a novel N-terminal domain encoded by rASV1702 src was shown to be involved in but not sufficient for full protection. These data indicate that determinants on or induced by rASV157 and rASV1702 can elicit a potent protection against the tumorigenic potential of RSV-encoded p60v-src.
The South Atlantic Convergence Zone: Intensity, Form, Persistence, and Relationships with Intraseasonal to Interannual Activity and Extreme Rainfall

Abstract The characteristics of intensity, geographical location, and persistence of the South Atlantic convergence zone (SACZ) during the austral summer are investigated. Intensity and spatial features of the SACZ are identified by performing a factor analysis of structural properties of outgoing longwave radiation (OLR) data. The first two leading factors explain 65% of the total variance of structural properties and characterize the SACZ according to intensity and location (oceanic versus continental). An index is constructed based on the magnitude of the factor scores to identify intense (weak) and oceanic (continental) SACZ. The intense SACZ category is associated with negative OLR anomalies over a large area of tropical South America, extending from the western Amazon to the Atlantic Ocean. The weak SACZ category is observed with positive OLR anomalies over tropical South America and negative OLR anomalies over southeastern South America. Oceanic and continental aspects of the SACZ are related to a .
Experimental result analysis for a generative probabilistic image retrieval model

The main conclusion from the metrics-based evaluation of video retrieval systems at TREC's video track is that non-interactive image retrieval from general collections using visual information only is not yet feasible. We show how a detailed analysis of retrieval results -- looking beyond mean average precision (MAP) scores on topical relevance -- gives significant insight in the main problems with the visual part of the retrieval model under study. Such an analytical approach proves an important addition to standard evaluation measures.
Reversed remodelling of dilated left sided cardiomyopathy after upgrading from VVIR to VVIR biventricular pacing.

AIMS To show reversed remodelling of the left ventricle and left atrium after upgrading to biventricular pacing in a patient with worsening heart failure due to VVIR pacing. To review the literature concerning reversed remodelling after upgrading to biventricular pacing. METHODS AND RESULTS In a 61-year-old man, with cardiomyopathy and worsening heart failure and mitral insufficiency following conventional pacing after His-bundle ablation, upgrading to biventricular pacing showed left sided reversed remodelling of the heart with decrease in mitral regurgitation in 6 months. The literature is reviewed by a Medline search between 1966-2001. CONCLUSION The occasionally observed progressive heart failure after right ventricular pacing following His-bundle ablation can be reversed by upgrading to VVIR biventricular pacing and under these circumstances upgrading should be considered.
Photooxidation of Tryptophan: O2( 1 Δg) versus Electron‐Transfer Pathway

Abstract— Tris (2,2'‐bipyridyl)ruthenium(II)chloride hexahydrate (Ru[bpy]32+) free in solution and adsorbed onto antimony‐doped SnO2 colloidal particles was used as a photosensitizer for a comparison of the O2(1Δg) and electron‐transfer‐mediated photooxidation of tryptophan (TRP), respectively. Quenching of excited Ru(bpy)32+ by O2(3σg−) in an aerated aqueous solution leads only to the formation of O2(1Δg) (φ4= 0.18) and this compound was used as a type II photosensitizer. Excitation of Ru(bpy)32+ adsorbed onto Sb/SnO2 results in a fast injection of an electron into the conduction band of the semiconductor and accordingly to the formation of Ru(bpy)32+ and was used for the sensitization of the electron‐transfer‐mediated photooxidation. The Ru(bpy)33+ is reduced by TRP with a bimolecular rate constant kQ= 5.9 × 108M−1 s−1, while O2(1Δg) is quenched by TRP with kt= 7.1 × 107M−1 s−1 (chemical + physical quenching). Relative rate constants for the photooxidation of TRP (kc) via both pathways were determined using fluorescence emission spectroscopy. With Np, the rate of photons absorbed, being constant for both pathways we obtained kc= (372/Np) M−1 s−1 for the O2(1Δg) pathway and kc≥ (25013/Np) M−1 s−1 for the electron‐transfer pathway, respectively. Thus the photooxidation of Trp is more than two orders of magnitude more efficient when it is initiated by electron transfer than when initiated by O2(1Δg).
Structural and functional abnormalities of retinal ribbon synapses due to Cacna2d4 mutation.

PURPOSE In a spontaneous mutant substrain of C57BL/10 mice, severely affected retinal ribbon-type synapses have been described. The retinopathy was accompanied by a substantial loss in the activities of the second-order neurons. Rod photoreceptor responses were maintained with reduced amplitude, whereas cone activities were absent. This study was conducted to identify the genetic defect underlying this hitherto unknown autosomal recessive cone-rod dysfunction. METHODS Genome-wide linkage analysis and screening of positional candidate genes were used to identify the causative mutation. Tissue-specific transcriptional activity of the defective gene was determined by Northern blot analysis and RT-PCR approaches. The number of cone photoreceptors was estimated by immunohistochemistry. RESULTS The mutation was localized to a 275-kb region of chromosome 6. Within this candidate interval, a homozygous frameshift mutation (c.2367insC) was identified in the Cacna2d4 gene of affected animals. This gene codes for an L-type calcium channel auxiliary subunit of the alpha2delta type. The mutation introduces a premature stop codon that truncates one third of the predicted Cacna2d4 protein. A severe reduction in Cacna2d4 transcript levels observed in mutant retinas probably results in the lack of Cacna2d4 protein. The mutation leads to significant loss of rods, whereas the number of cone cells remains unaffected until 6 weeks of age. CONCLUSIONS The Cacna2d4 mutation underlies a novel channelopathy leading to cone-rod dysfunction in the visual system of mice and provides a new candidate gene for human retinal disorders including night blindness, retinitis pigmentosa, and cone-rod dystrophies.
Changes in the Clinical Outcomes of Variceal Bleeding in Cirrhotic Patients: A 10-Year Experience in Gangwon Province, South Korea

Background/Aims Variceal rupture is one of the main causes of mortality in cirrhotic patients. However, there are limited data on the long-term outcomes of variceal bleeding. Methods We investigated the incidence and mortality of variceal bleeding at three endoscopic centers in Gangwon province during 3 periods (August 1996 to July 1997, August 2001 to July 2002, and August 2006 to July 2007). Results A total of 1,704 upper gastrointestinal (GI) bleedings occurred during the study periods. Peptic ulcers were found in 825 patients (48.5%), and variceal ruptures were found in 607 patients (35.6%). The variceal bleeding rate did not decrease in each period (26.0% vs 43.7% vs 33.9%, respectively). In the variceal bleeding group, the 6-week mortality rate steadily and significantly decreased (15.5% vs 10.8% vs 6.4%, respectively, p=0.027). In addition, the mortality rate was significantly higher in the variceal bleeding group than in the non-variceal bleeding group (10.4% vs 2.0%, p<0.001; odds ratio, 5.659; 95% confidence interval, 3.445 to 9.295). Conclusions Variceal bleeding was still the major cause of upper GI bleedings and did not decrease in prevalence over the 10-year period in Gangwon province, South Korea. However, the mortality rate of variceal bleeding decreased significantly.
Automated 3D Face Reconstruction from Multiple Images Using Quality Measures

Automated 3D reconstruction of faces from images is challenging if the image material is difficult in terms of pose, lighting, occlusions and facial expressions, and if the initial 2D feature positions are inaccurate or unreliable. We propose a method that reconstructs individual 3D shapes from multiple single images of one person, judges their quality and then combines the best of all results. This is done separately for different regions of the face. The core element of this algorithm and the focus of our paper is a quality measure that judges a reconstruction without information about the true shape. We evaluate different quality measures, develop a method for combining results, and present a complete processing pipeline for automated reconstruction.
Protein leverage affects energy intake of high-protein diets in humans.

BACKGROUND The protein leverage hypothesis requires specific evidence that protein intake is regulated more strongly than energy intake. OBJECTIVE The objective was to determine ad libitum energy intake, body weight changes, and appetite profile in response to protein-to-carbohydrate + fat ratio over 12 consecutive days and in relation to age, sex, BMI, and type of protein. DESIGN A 12-d randomized crossover study was performed in 40 men and 39 women [mean ± SD age: 34.0 ± 17.6 y; BMI (in kg/m(2)): 23.7 ± 3.4] with the use of diets containing 5%, 15%, and 30% of energy from protein from a milk or plant source. RESULTS Protein-content effects did not differ by age, sex, BMI, or type of protein. Total energy intake was significantly lower in the high-protein (7.21 ± 3.08 MJ/d) condition than in the low-protein (9.33 ± 3.52 MJ/d) and normal-protein (9.62 ± 3.51 MJ/d) conditions (P = 0.001), which was predominantly the result of a lower energy intake from meals (P = 0.001). Protein intake varied directly according to the amount of protein in the diet (P = 0.001). The AUC of visual analog scale appetite ratings did not differ significantly, yet fluctuations in hunger (P = 0.019) and desire to eat (P = 0.026) over the day were attenuated in the high-protein condition compared with the normal-protein condition. CONCLUSIONS We found evidence to support the protein leverage hypothesis in that individuals underate relative to energy balance from diets containing a higher protein-to-carbohydrate + fat ratio. No evidence for protein leverage effects from diets containing a lower ratio of protein to carbohydrate + fat was obtained. It remains to be shown whether a relatively low protein intake would cause overeating or would be the effect of overeating of carbohydrate and fat. The study was registered at clinicaltrials.gov as NCT01320189.
Foreign direct investment and host country productivity : the American automotive component industry in the 1980 s

Received: September 2001 Revised: June 2002 Accepted: August 2002 Online publication date: 27 March 2003 Abstract Although several studies have shown that inward foreign direct investment (FDI) often leads to greater host country productivity, researchers have yet to determine the relative importance of direct technology transfer and competitive pressure. To assess the relative importance of the two channels, we examine the US auto-component industry between 1979 and 1991. During this period, Japanese automobile assemblers began to produce vehicles in North America, and began to purchase inputs from US auto-component manufacturers. Those US manufacturers that sold components to Japanese transplants would be the direct recipients of any technologies transferred from the Japanese. Although we find that the direct investment by Japanese assemblers was associated with overall productivity improvement in the US auto-component industry, we find little evidence of direct technology transfer. The productivity growth of US suppliers affiliated with Japanese assemblers was no greater than that of other, non-affiliated US suppliers. Further, we find that the Japanese assemblers tended to purchase components from less productive US suppliers and, moreover, that low-productivity suppliers that sold goods to Japanese assemblers had a higher survival rate than low-productivity suppliers that did not sell to Japanese firms. The results suggest that increased competitive pressure in the auto-sector was the main cause of overall productivity improvement, at least during the initial stages of FDI of the 1980s. Journal of International Business Studies (2003) 34, 199–218. doi:10.1057/ palgrave.jibs. 8400017
An efficient and effective algorithm for density biased sampling

In this paper we describe a new density-biased sampling algorithm. It exploits spatial indexes and the local density information they preserve, to provide improved quality of sampling result and fast access to elements of the dataset. It attains improved sampling quality, with respect to factors like skew, noise or dimensionality. Moreover, it has the advantage of efficiently handling dynamic updates, and it requires low execution times. The performance of the proposed method is examined experimentally. The comparative results illustrate its superiority over existing methods.
Serum Amyloid A as a Predictor of Coronary Artery Disease and Cardiovascular Outcome in Women: The National Heart, Lung, and Blood Institute–Sponsored Women’s Ischemia Syndrome Evaluation (WISE)

Background—Serum amyloid-&agr; (SAA) is a sensitive marker of an acute inflammatory state. Like high-sensitivity C-reactive protein (hs-CRP), SAA has been linked to atherosclerosis. However, prior studies have yielded inconsistent results, and the independent predictive value of SAA for coronary artery disease (CAD) severity and cardiovascular events remains unclear. Methods and Results—A total of 705 women referred for coronary angiography for suspected myocardial ischemia underwent plasma assays for SAA and hs-CRP, quantitative angiographic assessment, and follow-up evaluation. Cardiovascular events were death, myocardial infarction, congestive heart failure, stroke, and other vascular events. The women’s mean age was 58 years (range 21 to 86 years), and 18% were nonwhite. SAA and hs-CRP were associated with a broad range of CAD risk factors. After adjustment for these risk factors, SAA levels were independently but moderately associated with angiographic CAD (P =0.004 to 0.04) and highly predictive of 3-year cardiovascular events (P <0.0001). By comparison, hs-CRP was not associated with angiographic CAD (P =0.08 to 0.35) but, like SAA, was strongly and independently predictive of adverse cardiovascular outcome (P <0.0001). Conclusions—Our results show a strong independent relationship between SAA and future cardiovascular events, similar to that found for hs-CRP. Although SAA was independently but moderately associated with angiographic CAD, this association was not found for hs-CRP. These results are consistent with the hypothesis that systemic inflammation, manifested by high SAA or hs-CRP levels, may promote atherosclerotic plaque destabilization, in addition to exerting a possible direct effect on atherogenesis.