Miracle-dz/UniFAR-FTUs huggingface dataset:
MR imaging findings in children with merosin-deficient congenital muscular dystrophy.

BACKGROUND AND PURPOSE Our purpose was to determine the brain MR imaging characteristics of merosin-deficient congenital muscular dystrophy in children. METHODS We reviewed the MR imaging findings of the brain in three children with known merosin-deficient congenital muscular dystrophy to determine the presence of any cerebral or cerebellar abnormalities of development or abnormalities of the white matter. RESULTS In all three patients, there was normal formation of the cerebrum, the cerebellum, and no evidence of neuronal migration anomalies. All three patients had abnormal white matter in the cerebrum, with sparing of the corpus callosum, internal capsule, cerebellum, and brain stem. CONCLUSION MR imaging of the brain in children with merosin-deficient congenital muscular dystrophy reveals a consistent pattern of white matter abnormality. We postulate that disruption of the blood-brain barrier associated with merosin deficiency leads to increased water content, resulting in abnormal white matter signal intensity.
Proteomic analyses identify a diverse array of nuclear processes affected by small ubiquitin-like modifier conjugation in Arabidopsis

The covalent attachment of SUMO (small ubiquitin-like modifier) to other intracellular proteins affects a broad range of nuclear processes in yeast and animals, including chromatin maintenance, transcription, and transport across the nuclear envelope, as well as protects proteins from ubiquitin addition. Substantial increases in SUMOylated proteins upon various stresses have also implicated this modification in the general stress response. To help understand the role(s) of SUMOylation in plants, we developed a stringent method to isolate SUMO-protein conjugates from Arabidopsis thaliana that exploits a tagged SUMO1 variant that faithfully replaces the wild-type protein. Following purification under denaturing conditions, SUMOylated proteins were identified by tandem mass spectrometry from both nonstressed plants and those exposed to heat and oxidative stress. The list of targets is enriched for factors that direct SUMOylation and for nuclear proteins involved in chromatin remodeling/repair, transcription, RNA metabolism, and protein trafficking. Targets of particular interest include histone H2B, components in the LEUNIG/TOPLESS corepressor complexes, and proteins that control histone acetylation and DNA methylation, which affect genome-wide transcription. SUMO attachment site(s) were identified in a subset of targets, including SUMO1 itself to confirm the assembly of poly-SUMO chains. SUMO1 also becomes conjugated with ubiquitin during heat stress, thus connecting these two posttranslational modifications in plants. Taken together, we propose that SUMOylation represents a rapid and global mechanism for reversibly manipulating plant chromosomal functions, especially during environmental stress.
Asset Pricing Under a Subset of Linear Risk Tolerance Functions and Log-Normal Market Returns

The Capital Asset Pricing Model (CAPM) developed and popularized by Treynor [27], Sharpe [26], Lintner [16], Mossin [19], and Fama [6] is of the form where E is the expected return at time t for firm i (conditional on information available at time t); the subscript m denotes the analogous market variable; rf is the risk-free rate; and . Black [2] has developed a similar form with expected returns from a zero beta portfolio, , assuming the role of the risk-free rate.
Falling between the Cracks: Privatisation and Corporate Governance in the European Telecom Industry

Newly privatised firms can often fall between the cracks, being at the same time more risky than SOEs and less profitable/competitive than private firms. A well-designed corporate governance regime is an important tool to make the transition period shorter and less painful. After reviewing the partially or wholly privatised European telecom sector, the paper proposes eight lessons for policy makers in charge of designing privatisation. First, where the State remains an important owner after partial privatisation, it should organise its shareholding function to pursue exclusively shareholder value objectives. Second, any privatisation-related asymmetries between control and cash-flow rights among shareholders should be limited in time and scope. Third, while some minority shareholder power, such as direct shareholder nomination and cumulative voting are welcome, the board needs to develop its own cohesiveness and culture. Fourth, the board should be actively and effectively involved in the development and validation of the company's strategy and the control of major transactions. Fifth, the privatised firms should strive to list in a market with high and credible disclosure requirements. Sixth, privatised firms should focus on developing a disclosure culture, especially as regards non-financial disclosure. Seventh, the board should conduct a regular, thorough and independent evaluation of the CEO, based on a set of criteria and yearly objectives agreed at the beginning of each exercise.
Low Vision Rehabilitation Plans Comparing Two Intake Methods

Purpose. This qualitative study investigates possible differences in identified rehabilitation needs indicated by the usual intake procedures at a Multidisciplinary Rehabilitation Center (MRC) for visually impaired persons compared with those indicated by the use of a structured Dutch version [based on the International Classification of the Disability, Functioning and Health (ICF)] of the Activity Inventory (D-AI). Methods. Twenty patients who enrolled at the MRC received a D-AI assessment by telephone, in addition to the usual intake. All patients received usual care, based on rehabilitation needs identified by the usual intake procedure at the MRC. Rehabilitation needs identified at the MRC were obtained from patient files retrospectively and were compared with rehabilitation needs identified by the D-AI. Results. The mean number of rehabilitation needs reported in the patient files was 6.9 (±5.1) vs. 24.0 (±11.2) using the D-AI. Only 22.6% (±14.3) of the rehabilitation needs identified by the D-AI were present in the patient files; 79.3% (±28.2) of the rehabilitation needs reported in the patient files were identified by the D-AI. Overall agreement corrected for chance between both intake methods revealed a fair Cohen kappa of 0.27. Conclusions. At the MRC, more needs were revealed using the D-AI compared with the usual intake procedure. The systematic character of the D-AI prevents important topics being overlooked. In the usual intake, it was not clear whether needs were investigated from the patient's perspective. This may hamper (medical) communication and shared decision making about the rehabilitation program that needs to be followed. Moreover, using the unstructured information from the patient files makes it difficult to evaluate rehabilitation outcomes. With the D-AI, although an extensive overview of rehabilitation needs is produced, it remains difficult to focus on the most relevant needs. However, after assessment with the D-AI, all aspects of the ICF, Disability, and Health scheme can be discussed in a process of shared decision making, which leads to the final determination of rehabilitation goals.
360‐degree feedback PLUS: Boosting the power of co‐worker ratings for executives

As useful as co-worker ratings can be, all too often managers are given feedback and nothing changes. This is not surprising, since habits die hard. But, what if, to stand a better chance of having an impact, the power of co-worker ratings were boosted? This article describes several possible boosters: follow-through, using verbal descriptions along with ratings; collecting data on motivation as well as behavior; getting a historical perspective on the individual's current managerial behavior; and gathering data on the individual outside of work. A caveat: The more data that are collected and the greater the depth of these data, the greater the responsibility of those who conduct such an exercise. Feedback PLUS means commitment PLUS. This is all the more true when all the boosters are used in concert—an intervention the author and his colleagues have employed with executives. © 1993 by John Wiley & Sons, Inc.
Productive but Not Valued: The Russian State Duma, 1994-2001

MOST COMMENTATORS VIEW RUSSIAN POLITICS as structured to a very large extent by the executive and not by parliament. ' On the one hand, the Russian constitution is said to place the State Duma, the lower house of the Russian Federal Assembly, de jure in a position of weakness. The president's combined legislative powers to veto parliamentary bills2 and to issue decrees permit the head of state to rule by decree if he or she commands the support of just one-third of either house of the Federal Assembly (Parrish, 1998, p. 79). For this reason Holmes describes the Russian constitutional regime as 'superpresidential', the corollary of which is 'fig-leaf parliamentarianism' (Holmes, 1994, p. 124). On the other hand, analysts point to the de facto limitations on parliament's capacity to act. The polarised and fragmented character of the lower chamber is said to contribute to parliament's weakness (Holmes, 1994, p. 124), and is linked by other studies to the Duma's dysfunctional organisation (Ostrow, 1998). As Ostrow argues, the Duma's unlinked dual-channel design and poor co-ordination of legislative committees and parliamentary parties '. renders the Russian legislature unable to manage internal conflict on legislative issues . ' and makes legislative gridlock the norm in the Duma (Ostrow, 1998, pp. 793, 809; see also Ostrow, 2000, chapter 5). The overwhelming majority of commentators regard 'fig-leaf parliamentarism', or a situation in which the State Duma '. does not in any real sense share sovereignty with the chief executive' (Huskey, 1999, p. 180; see also Huskey, 2001), as deeply undesirable. A de jure and de facto weak assembly exposes Russia to the risk of becoming a delegative democracy in which parliamentary checks and balances are largely inoperative, and accountability and representation are flouted by an unrestrained president between elections (O'Donnell, 1998; Parrish, 1998). Yet several analysts have challenged the view that the Duma is a powerless institution. In their study of law making in Russia between 1994 and 1997 Remington, Smith & Haspel (1998, p. 320) conclude that, 'contrary to popular impressions, Russia has been governed by more than presidential decree since late 1993'. Moreover, their evidence on parliament's involvement in the policy-making process indicates 'a somewhat greater role for parliament than the 1993 constitution . would suggest' (p. 320; see also Remington, 2001, pp. 217-231). These contrasting views of parliament's role deserve closer scrutiny because they bear on our understanding of both the governmental process in Russia and the
Asthma prevalence, family size, and birth order.

BACKGROUND Asthma prevalence may be reduced in large families. The hygiene hypothesis suggests that older siblings protect their younger siblings from asthma through a modulating effect on the still-maturing immune system. If the hygiene hypothesis is correct, asthma prevalence should be inversely related to birth order. The objective of this study was to examine the relationship between asthma prevalence, and family size and birth order. METHODS The medical records of 531,116 Israeli military conscripts were reviewed. The association between number of children in the family and the prevalence of asthma, and between birth order and the prevalence of asthma was assessed. Odds ratios for asthma by birth order and family size, adjusted for each other, were calculated. RESULTS Asthma was diagnosed in 26,833 male subjects (8.6%) and 15,079 female subjects (6.9%). Asthma prevalence was inversely related to the number of children in the family (p < 0.001). Among subjects who were the only child in the family, the prevalence of asthma was 7.3%. The prevalence increased to 8.95% among subjects from families with three siblings, and then progressively decreased as the number of siblings increased, and reached a trough of 0.58% in conscripts from families of 15 to 20 siblings. Asthma prevalence was similar for all birth orders. CONCLUSIONS In families with four or more children, asthma prevalence is inversely related to the number of children in the family. Asthma prevalence is similar for all birth orders. The similar asthma prevalence for all birth orders challenges the hygiene hypothesis as the mechanism for the decreased asthma prevalence in large families.
Pheromone‐regulated target genes respond differentially to MAPK phosphorylation of transcription factor Prf1

Pheromone signalling during mating is essential for pathogenicity of Ustilago maydis. The activity of the key transcription factor Prf1 is controlled at the transcriptional level and post‐translationally by mitogen‐activated protein kinase (MAPK) and protein kinase A (PKA) phosphorylation. However, the precise contribution of these regulatory mechanisms to the transcriptional output is unknown. Here, we genetically dissected the three levels of Prf1 regulation. We performed transcriptional profiling of respective mutants to identify and classify targets. This approach revealed that transcriptional regulation of prf1 had only minor influence on target gene expression stressing the importance of post‐translational control. PKA regulation of Prf1 was sufficient to control expression of nine pheromone‐responsive genes including the major transcription factor regulating pathogenicity. MAPK regulation was necessary for the pheromone response of a set of 57 genes. In 35 cases, pheromone responsiveness was completely lost, while in the remaining 22 cases regulation was alleviated. This indicated a novel level of complexity in MAPK signalling suggesting that target genes respond differentially to MAPK phosphorylation of the respective transcription factors.
Communicating persuasive messages through slow food festivals

As an active social and political philosophy, slow food aims to change the world, encouraging the public to opt for healthier food and reinvigorate traditional recipes and practices. Persuading people of this message is achieved through a range of methods, including media (particularly television, magazines and the Internet), restaurants, food retailers and food-themed festivals. In this article, we are examining how the latter are used to promote the persuasive messages of slow food. We use a comparative case study approach based on five international examples of festivals branded as ‘slow’ or that incorporate slow food elements into their theme or event concept. Two of the festivals are in Italy, where the slow food movement began and remains popular. The other three are from Australia and New Zealand, the countries which have embraced slow food, although lacking the entrenched cultural heritage of distinct regional cuisines. The event web sites present detailed and persuasive messages about what slow food is, how it is aiming to change patterns of behaviour and the rejection of modern fast-food cultures. Communication via these web sites is both multi-storeyed and multi-storied. There are complex uses of imagery and metaphor, the usage of well-known media food personalities as champions, and the strong marketing of slow food as authentic, artisanal and high-status.
Association between Neopterin and Carotid Intima-Media Thickness in Hemodialysis Patients

Background: Atherosclerotic lesions are heavily infiltrated by macrophages. Neopterin can be used as a marker of the activity of macrophages. Serum neopterin levels were elevated in non-renal patients with atherosclerosis. The intima-media thickness (IMT) of the carotid arteries in hemodialysis patients was significantly higher than in control subjects. In this study, we measured serum neopterin levels in hemodialysis patients and evaluated a possible correlation between neopterin levels and carotid IMT. Patients and Methods: Thirty-seven hemodialysis patients (26 male/11 female, mean age 47 ± 15 years) and 12 healthy subjects (8 male/4 female, mean age 43 ± 10 years) were included in this study. Serum neopterin levels were measured by using a commercial ELISA kit. Carotid IMT of the subjects were measured by high-resolution B-mode ultrasonography. Results: Carotid IMT values were 1.04 ± 0.29 and 0.77 ± 0.25 mm in hemodialysis patients and healthy controls, respectively (p < 0.01). Serum neopterin levels were 110.9 ± 19.1 and 3.8 ± 2.3 ng/ml in hemodialysis patients and healthy controls, respectively (p < 0.01). Serum neopterin levels were 103.2 ± 21.3 ng/ml in hemodialysis patients with IMT <1 mm (n = 15), and 116.7 ± 15.4 ng/ml in hemodialysis patients with IMT ≧1 mm (n = 22) (p < 0.05). Moreover, there was a significant correlation between serum neopterin levels and carotid IMT (p < 0.05, r = 0.363). Conclusion: Our findings suggest that neopterin could be associated with the severity of carotid atherosclerosis in hemodialysis patients.
Identification of hot spot and safe zones of ambient air quality in North Maharashtra region, India

The continuous infrastructural and economic development exerted pressure on the environmental conditions of the North Maharashtra region. The air quality of North Maharashtra region was analysed for the identification of hot spots and safe zones of ambient air quality. The result shows air pollution at some part of North Maharashtra region. The levels of particulate matter were above the prescribed limits especially at Bhusawal, Dhule and Jalgaon Urban centres. The traces of polyaromatic hydrocarbons were also observed in the urban centres of study area. The binary ratios of the persistent prevailing polycyclic aromatic hydrocarbon (PAH)'s show great correlation with the vehicular exhaust in the study region. The higher levels of pollutants in the study region were recorded in the post-monsoon season. The present study is first of its kind in the North Maharashtra region and an attempt has been made to analyse the anthropogenic sources and concentration of air pollutants in the area. The air pollutants reduce the defence system of human body and lead into severe health consequences like damage of lung tissues with escalation in the existing respiratory and cardiovascular diseases.
Information Privacy and Marketing: What the U.S. Should (and Shouldn't) Learn from Europe

The U.S. and Europe exhibit very different approaches to information privacy—a condition of limited access to identifiable information about individuals—from both regulatory and managerial perspectives. Grounded in different cultural values and assumptions about the meaning of privacy (a 99human rights" issue in Europe versus a contractual issue in the U.S.), these differences have led to regulatory and managerial conflicts. In this article, the differences between the two approaches are explored. U.S. corporations would be well served to embrace some of the premises of the European perspective. However, the U.S. would be poorly served by the creation of a federal regulatory structure such as some commonly found in Europe.
Information processing profiles of internalizing and externalizing behavior problems: evidence from a population-based sample of preadolescents.

BACKGROUND The present study explores the relationships between several information processing capacities and internalizing and externalizing behavior problems in a general population sample of 10- to 12-year olds (N = 2,037 51% girls). METHODS Parent-reported behavior problems as assessed by the Child Behavior Checklist were used to form four groups of children with 1) neither internalizing nor externalizing problems (n = 1,470), 2) only internalizing problems (n = 237), 3) only externalizing problems (n = 182), and 4) both internalizing and externalizing problems (n = 148). These groups were compared on measures of speed and accuracy from the Amsterdam Neuropsychological Tasks program reflecting the efficiency of several input-, central cognitive-, and output-related information processing capacities. RESULTS Children with both internalizing and externalizing problems demonstrated the least efficient performance, followed by children with only externalizing problems, whereas children with only internalizing problems did not differ from children without problems. More specifically, response variability and the ability to maintain and quickly compare information in working memory were found to be related to the severity of problem behavior. The ability to inhibit prepotent responses was related to the type of problem behavior, discriminating between the children with only internalizing problems and only externalizing problems. However, this latter capacity no longer differentiated when controlling for IQ. No differences were found between boys and girls. CONCLUSIONS The results suggest that, in general, cognitive processing deficits are more strongly related to the degree than to the type of maladaptive behavior. Furthermore, response variability and working memory may serve as potential markers for identifying high-risk children and response inhibition as an indicator of the type of maladaptive behavior.
The Calculation of Minimum Margin

The calculation of margin for investor's option accounts is a complex and costly problem for brokerage houses. The existing procedures usually involve a heuristic requiring sequential computations. These are shown to be inefficient and suboptimal. A simple transportation formulation is presented which permits a direct computation of minimum margin and shows considerable savings when compared with existing heuristic procedures.
The &agr;2‐Adrenergic Agonists Clonidine and Guanfacine Produce Tonic and Phasic Block of Conduction in Rat Sciatic Nerve Fibers

&NA; To determine whether &agr;2‐adrenergic agonists inhibit impulse conduction, clonidine and guanfacine were applied to rat sciatic nerve fibers studied in vitro. Clonidine and guanfacine produced concentration‐dependent, tonic inhibition of compound action potentials in large, myelinated A&agr; fibers. The 50% effective concentration (EC50) of clonidine measured 2.0 ± 0.8 mM (mean ± standard deviation); the EC50 of guanfacine measured 1.2 ± 0.2 mM. Clonidine was also less potent than guanfacine at phasic block of A&agr; compound action potentials examined at 10 Hz. Both drugs inhibited tonic impulse conduction in C fibers in a concentration‐dependent, reversible fashion, and produced greater inhibition of C fiber than A&agr; compound action potentials at all drug concentrations. Again, clonidine appeared to inhibit C fiber compound action potentials (EC50 = 0.45 ± 0.01 mM) with less potency than guanfacine (EC50 = 0.17 ± 0.06 mM). We conclude that clonidine and guanfacine, unlike traditional local anesthetics, demonstrate a tendency toward steady‐state differential nerve block wherein C fibers are blocked to a greater extent than A&agr; fibers. (Anesth Analg 1993;76:295‐301)