Miracle-dz/UniFAR-FTUs huggingface dataset:
Neuroimaging Correlates of Frontotemporal Dementia Associated with SQSTM1 Mutations.

BACKGROUND Frontotemporal lobar degeneration (FTLD) is a progressive dementia characterized by focal atrophy of frontal and/or temporal lobes caused by mutations in the gene coding for sequestosome 1 (SQSTM1), among other genes. Rare SQSTM1 gene mutations have been associated with Paget's disease of bone, amyotrophic lateral sclerosis, and, more recently, frontotemporal lobar degeneration (FTLD). OBJECTIVE The aim of the study was to determine whether a characteristic pattern of grey and white matter loss is associated with SQSTM1 dysfunction. METHODS We performed a voxel-based morphometry (VBM) study in FTD subjects carrying SQSTM1 pathogenic variants (FTD/SQSTM1 mutation carriers; n = 10), compared with FTD subjects not carrying SQSTM1 mutations (Sporadic FTD; n = 20) and healthy controls with no SQSTM1 mutations (HC/SQSTM1 noncarriers; n = 20). The groups were matched according to current age, disease duration, and gender. RESULTS After comparing FTD/SQSTM1 carriers with Sporadic FTD, a predominantly right cortical atrophy pattern was localized in the inferior frontal, medial orbitofrontal, precentral gyri, and the anterior insula. White matter atrophy was found in both medial and inferior frontal gyri, pallidum, and putamen. FTD/SQSTM1 carriers compared with HC/SQSTM1 noncarriers showed atrophy at frontal, temporal, and parietal lobes of both hemispheres whereas the MRI pattern found in Sporadic FTD compared with controls was frontal and left temporal lobe atrophy, extending toward parietal and occipital lobes of both hemispheres. CONCLUSIONS These results suggest that fronto-orbito-insular regions including corticospinal projections as described in ALS are probably more susceptible to the damaging effect of SQSTM1 mutations delineatinga specific gene-linked atrophy pattern.
DIFFICULTIES IN MEASURING THE COST OF POOR OUALITY

Reducing a company's non‐value creating costs — the cost of poor quality — is one of the best ways of increasing profitability and competitiveness. The potential is considerable. The cost of poor quality usually corresponds to between 10% and 30% of total turnover. But to realize the benefits requires detailed information about the magnitude and distribution of these costs that is often difficult to obtain.
Watching amyloid fibrils grow by time-lapse atomic force microscopy.

Late-onset diabetes is typically associated with amyloid deposits of fibrillar amylin in the pancreatic islets. Aqueous synthetic human amylin spontaneously forms polymorphic fibrils in vitro, and this system was used to examine the dynamics of fibril assembly. By time-lapse atomic force microscopy (AFM), the growth of individual amylin fibrils on a mica surface was observed over several hours. Prominent was the assembly of a protofibril with an elongation rate in these experiments of 1.1(+/-0.5) nm/minute. The assembly of higher order polymorphic fibrils was also observed. Growth of the protofibrils was bidirectional, i.e. it occurred by elongation at both ends. This ability of AFM to continuously monitor growth, directionality, and changes in morphology for individual fibrils, provides a significant advantage over spectroscopy-based bulk methods which average the growth of many fibrils and typically require 100 to 1000-fold more protein. The time-lapse AFM procedure used for human amylin here is thus likely to be applicable to fibril formation from other amyloid proteins and peptides.
Education, Social Cohesion and Economic Growth

Analysis of the contribution of education to growth through its role in promoting a common culture indicates that when different cultural groups separately determine the social content of their school curricula excessive polarization can result, with less than optimal growth. The optimal trajectory involves a gradual, reciprocal convergence of school curricula towards the middle ground. This may be difficult to implement in a political context in which all agents are identified with one group or another. When curricula are determined by legislative bargaining, centralization of schooling may result in overly rapid homogenization in some cases, and - perhaps surprisingly - excessive polarization in others.
Design Engineering in Virtual Environments

The current Space Shuttle Orbital Maneuvering System (OMS) and the related Reaction Control System (RCS) make use of hydrazine as a propellant. Hydrazine, while high in energy content, is both corrosive and poisonous. As a result, its use in the OMS/RCS dictates that extreme caution be used in both the initial charging of the systems before launch and in their safetying after landing. Not only does the use of hydrazine increase the risk for those preparing and maintaining the Space Shuttle, but the procedures required for personnel safety during these processes add significant time to the preparation of each Space Shuttle for launch. As a result, the Space Shuttle Upgrade Board approved the design of a new OMS/RCS utilizing a non-toxic propellant. At the same time, this engineering design effort was recognized as an ideal opportunity to assess the overall maintainability of the OMS/RCS and to incorporate, where possible, design and vehicle integration features that would significantly reduce the personnel and time requirements for processing these systems between flights. In an effort to reduce the overall engineering design time and costs, a partnership between the Propulsion Division and the Automation, Robotics, and Simulation Division of the NASA/Johnson Space Design Engineering in Virtual Environments R. Bowen Loftin
Two cases of thiopurine methyltransferase (TPMT) deficiency--a lucky save and a near miss with azathioprine.

BACKGROUND The association between thiopurine methyltransferase (TPMT) deficiency and myelosuppression with azathioprine is well recognized. Two cases are presented that illustrate the very different outcomes that may occur with azathioprine in patients with TPMT deficiency, which affects 0.3-0.6% of caucasians. CASE 1: The first patient's TPMT deficiency was identified following hospitalization for pancytopenia attributed to azathioprine. CASE 2: The second patient was identified as deficient early in treatment and myelosuppression was avoided by treating with a greatly decreased dose (25 mg per week). CONCLUSIONS Testing for TPMT ideally should be performed in every patient commencing a thiopurine drug.
Mechanism for Phosphorylation-induced Activation of the Phagocyte NADPH Oxidase Protein p47 phox

Activation of the superoxide-producing phagocyte NADPH oxidase requires interaction between p47 phox and p22 phox , which is mediated via the SH3 domains of the former protein. This interaction is considered to be induced by exposure of the domains that are normally masked by an intramolecular interaction with the C-terminal region of p47 phox . Here we locate the intramolecular SH3-binding site at the region of amino acid residues 286–340, where Ser-303, Ser-304, and Ser-328 that are among several serines known to become phosphorylated upon cell stimulation exist. Simultaneous replacement of the three serines in p47 phox with aspartates or glutamates, each mimicking phosphorylated residues, is sufficient for disruption of the intramolecular interaction and resultant access to p22 phox . The triply mutated proteins are also capable of activating the NADPH oxidase without in vitro activators such as arachidonate under cell-free conditions. In a whole-cell system where expression of the wild-type p47 phox reconstitutes the stimulus-dependent oxidase activity, substitution of the kinase-insensitive residue alanine for Ser-328 as well as for Ser-303/Ser-304 leads to a defective production of superoxide. These findings suggest that phosphorylation of the three serines in p47 phox induces a conformational change to a state accessible to p22 phox , thereby activating the NADPH oxidase.
A viral vector expressing hypoxia-inducible factor 1 alpha inhibits hippocampal neuronal apoptosis

Hypoxia-inducible factor 1 (HIF-1) attenuates amyloid-beta protein neurotoxicity and decreases apoptosis induced by oxidative stress or hypoxia in cortical neurons. In this study, we constructed a recombinant adeno-associated virus (rAAV) vector expressing the human HIF-1α gene (rAAV-HIF-1α), and tested the assumption that rAAV-HIF-1α represses hippocampal neuronal apoptosis induced by amyloid-beta protein. Our results confirmed that rAAV-HIF-1α significantly reduces apoptosis induced by amyloid-beta protein in primary cultured hippocampal neurons. Direct intracerebral rAAV-HIF-1α administration also induced robust and prolonged HIF-1α production in rat hippocampus. Single rAAV-HIF-1α administration resulted in decreased apoptosis of hippocampal neurons in an Alzheimer's disease rat model established by intracerebroventricular injection of aggregated amyloid-beta protein (25–35). Our in vitro and in vivo findings demonstrate that HIF-1 has potential for attenuating hippocampal neuronal apoptosis induced by amyloid-beta protein, and provides experimental support for treatment of neurodegenerative diseases using gene therapy.
URBAN WATER SUPPLY: A SECOND LOOK,

Abstract : The WATER SUPPLY volume discerned a consistent pattern of under-pricing and over-building. Rational reallocation of existing supplies, such as would occur if prices were raised in response to scarcity, is almost never even considered as an alternative to new construction. There are several reasons, aside from the electoral attractiveness of water projects. (1) Politically determined prices are inflexible. New York, for example, has not modified its price schedule since 1933. (Of course, had the city attempted to do something about prices in the 1965 shortage they would have run into conflict with the CEA 'guide-lines'). (2) A higher-order political rigidity makes it commonly impossible for one water jurisdiction to sell title or rights to another -- hence an incentive to rush into construction to nail down the supplies involved. (3) Certain errors in economic reasoning have played a role: Ignorance of the marginal principle, double-counting of benefits, the use of inappropriately low discount rates, etc. But these analytical errors have had much less practical significance than what might be called the non-analytical error. This is the belief, usually quite unconscious, that there are 'needs' or 'requirements' for water rather than economic demands.
Influences of Reduction Ratio on Mechanical Properties and Transformation Temperature of NiTi Drawn Wires

NiTi alloy wires are widely used in many applications for examples, orthodontic arch wires, mini screw implant, sensors, etc. Usually, wire drawing process is chosen to produce arch wires because of its high productivity as well as superior surface quality of the drawn wire surface. In the case of NITI arch wire, the cold work occurred during wire drawing operation also plays important role to control mechanical properties and transformation behavior of the wires. Thus, the purpose of this work is to study the influences of reduction ratio of cross sectional area and annealing temperature on transformation and mechanical behavior of the drawn wires. The wire materials used in the experiment are Ni51.4Ti48. 6 (atomic percentage) with initial wire diameter of 0.64 mm. The die tip is made of tungsten carbide which is inserted into the steel case made of tool steel. The reduction ratios of cross sectional area (%Re) are varied at three different levels, i.e., 10, 20 and 30%, respectively. In order to study the influences of post heat treatment temperature, the drawn wires are annealed for 3,600 sec at two different levels; 400 and 600C. The lubricant used in the experiments is sodium stearate powder. From the results, drawing force and surface roughness of the drawn wire strongly depend on %Re. in addition; tensile properties and phase transformation temperature noticeably depend on heat treatment temperature. Keyword: NiTi arch wire, Wire drawing, Reduction ratio, Heat treatment temperature, Phase Transformation temperature, Shape memory alloy
The Vampire State in Africa: The Political Economy of Decline in Ghana

Part I - theories of development and decline: development theory and the African experience the evolution of economic development theory and policy in Ghana a theory of economic decline. Part II - Ghana's economy in the 20th century: the colonial economy development in the post-war indigenous state the political economy of decline 1961-1983. Part III - the failure of cocoa: an economic analysis of the cocoa industry implications for managing the economic recovery in Ghana.
Knowledge-Based Entreprenuership in Poland

The importance of new firm creation in the post-Communist economies of East Central Europe (ECE) has been subject to extensive research. This paper focuses on an area of entrepreneurship which has received relatively little attention in the transition economy context but which is of particular importance for the modernization of the transition economies: knowledge-based entrepreneurship (KBE), or new firm creation in industries considered to be science-based or to use research and development (RD there is very little evidence of distributed, or open, innovation here. These firms have also managed to achieve their successes with little or no help from the state, and have, in most cases, had to rely on networks of families or friends (including those in local industrial clusters) in order to sustain and develop their business.
A method for using high density fusible Rose's metal with high precision machining in small animal imaging applications

We present a method for producing flexible and high precision solutions applicable to detector shielding and pinhole apertures using a fusible metal called Rose's metal. Custom Rose's metal parts have been manufactured to both shield detectors as well as incorporate platinum pinholes in a small animal imaging system based on BazookaSPECT technology dubbed InSPECT.
Tolterodinium (+)-(2R,3R)-hydrogen tartrate.

In the crystal structure of (R)-N,N-diisopropyl-3-(2-hydroxy-5-methylphenyl)-3-phenylpropylaminium (2R,3R)-hydrogen tartrate, C22H32NO+. C4H5O6-, the hydrogen tartrate anions are linked by O-H.O hydrogen bonds to form helical chains built from R(2)(2)(9) rings. These chains are linked by the tolterodine molecules via N-H.O and O-H.O hydrogen bonds to form separate sheets parallel to the (101) plane.
Lymphoid reconstitution after autologous PBSC transplantation with FACS-sorted CD34+ hematopoietic progenitors.

T-cell and B-cell reconstitution was studied in nine patients who received fluorescence activated cell sorter (FACS)-sorted autologous CD34+ hematopoietic progenitor cells (HPC). The mean numbers of T cells (CD3+), B cells (CD19+) and CD34+ HPC administered to each patient were . 004, . 002, and 1.8 x 10(6) cells/kg, respectively. After high-dose myeloablative chemotherapy (busulfan, cyclophosphamide, etoposide) CD34+) HPC were infused and lymphoid reconstitution was monitored using flow cytometry and reverse transcriptase-polymerase chain reaction (RT-PCR) amplification of VDJ T-cell receptor (TcR) sequences. Restoration of normal numbers of peripheral blood T cells and B cells among recipients of FACS-sorted CD34+ HPC was delayed compared to recipients of non-T-cell-depleted PBSC autografts. In both patient groups, the circulating T cells were primarily CD4-, CD8+, alphabeta TcR+, and CD45RO+, CD45RA- during the first 2 months after transplant. Subsequent increases in the frequency of CD45RA+ CD45RO- T cells occurred at 2 to 3 months after transplant, suggesting maturation of CD34+ hematopoietic progenitors to "naive" T cells. Analysis of the TcR repertoire after hematopoietic reconstitution demonstrated decreased diversity of Vbeta TcR expression associated with global decreases in the absolute number of total peripheral blood T cells and most Vbeta TcR+ subsets. Three of nine recipients of FACS-sorted CD34+ HPC demonstrated significant increases in the percentage of gammadelta+ peripheral T cells and CD5+ B cells at 3 to 9 weeks after transplantation, and all patients had transient oligoclonal expansions of T cells expressing specific Vbeta TcR. Transplantation with highly purified CD34+ HPC results in reduced diversity of the peripheral T-cell repertoire during the early post-transplant period compared with patients receiving unmanipulated or MoAb-depleted transplants.
A Swedish real estate stock market index, 1939–1998

Abstract This paper presents a new index for Sweden computed using a new time-series of 60 years of monthly returns of real estate stocks from 1939 to the present. The computation of the index is explained along with some general statistics. We find that the financial crisis of 1990-92 and the subsequent economic turmoil had a devastating effect on the real estate stock market. The returns are subject to kurtosis and skewness, especially during the last decade of the period. The Swedish real estate stock market was less sensitive than the Swedish stock market index. This new index offers a valuable data set for future research in financial economics as well as other disciplines.
Private Equity Fair Value Measurement: A Critical Perspective on IFRS 13

In this paper, we discuss IFRS 13 Fair Value Measurement with regard to private equity valuation. We raise issues on the fair value definition as an exit price and question the reliability of valuation techniques, which are categorised into Level 2 fair value hierarchy. Our paper questions whether fair value as defined by IFRS 13 is an appropriate measure for private equities and can contribute to enhancing transparency and comparability in financial statements, which is one of the purposes of the International Accounting Standards Board and the European Union Regulation 1606/2002.
Charge distributions of active biological pores

Charge distributions associated with active biological pores are calculated. The charge distributions include rings of reverse-sign, current-turning charges which surround the entrance and exit ends of the pore and cause current to turn into the pore at its entrance end (e.g., the intracellular side of a potassium pore) and to turn outward from the pore at its exit end (e.g., the extracellular side of a potassium pore). The magnitude and spatial extent of the rings depend directly on the magnitude of the current passing through the pore. In the absence of current, the rings disappear and when, for example, the pore's resistance becomes the dominant resistance in the system, the magnitude and spatial extent of the rings saturate. The reverse-sign, current-turning charges affect the local environment of proteins, such as ligand-binding proteins, known to reside adjacent to biological pores. The charges are thus expected to play a role in pore function such as by modulating the binding of charged species to the binding proteins.
An enterprise approach to the interactive objectives and constraints of Smart Grids

It is inevitable that envision of power systems will be based on the Smart Grid. On the other hand the energy crisis enforces utilizing renewable and clean energy through small-scale Microgrids. Interactions of these two new and growing technologies will require development of more complicated control strategies. In other words, while each Microgrid has to satisfy its own localized constraints and objectives, limitations and objectives enforced by the whole Smart Grid and/or Microgrids will require extended and more conservative constraints. The first part of the paper will provide a comprehensive review of Microgrid and Smartgrid control strategies. This review will cover clean energy stability analysis, generation control, generation forecasting, load forecasting, power management, load management, interchange analysis of Microgrids, multi-objective and multi-constraint optimization as well as load shedding on localized and global grids. Additionally, combination of some of the discussed control strategies will be discussed further through the application of an industrial tool on a real grid example.
Critical Literacy: Challenging the Assumptions of Mainstream Educational Theory

The focus of traditional educational theory and practice has long been on the acquisition of functional literacy skills. The discourse that emerges from this perspective has been linked to the maintenance of the structural inequalities that characterize the wider society. Educators who are seriously interested in schooling for social transformation in the interest of democracy and justice need to move beyond this limited vision. To challenge the traditional discourse of schooling, educators need to develop a new language. This paper argues that such a challenge can be initiated around the notion of critical literacy. By focusing on what it means to be a critically literate, democratic citizen, a collective vision of what it might be like to live in the best of all societies can be created and strategies developed to act upon that vision.
Expenditure Composition and Institutional Reform in Europe: A Policy Perspective

• "increasing the efficiency of the public sector, inter alia, by introducing mechanisms to assess the relationship between public funds and policy objectives and to help control spending" The purpose of this paper is to evaluate actual developments in EU Member States from the perspective of these agreed policy recommendations. Section 2 puts the discussion into perspective, by briefly reviewing the literature on the link between public expenditure and long-term growth. Sections 3 and 4 then evaluate developments in the composition of public expenditure. In doing so, Section 3 investigates the long-term trends while section 4 takes a detailed look at changes in the composition of public expenditure since the start of the Lisbon strategy in 2000. In discussing policy options, Section 5 then stresses the importance of budgetary institutions. It maintains that, although it would be difficult to establish a direct link between institutional reform and the degree to which expenditure has been directed towards productive items, the data indicate that all countries that have been at the forefront of institutional reform also managed to redirect their public expenditure towards public investment (as a proxy for physical capital) and education (as a proxy for human capital).
Federal Reserve Bank of Minneapolis Research Department Staff Report 284 Trade Theory and Trade Facts *

This paper quantitatively tests the " new trade theory " based on product differentiation, increasing returns, and imperfect competition. We employ a standard model, which allows both changes in the distribution of income among industrialized countries, emphasized by Helpman and Krugman (1985), and nonhomothetic preferences, emphasized by Markusen (1986), to effect trade directions and volumes. In addition, we generalize the model to allow changes in relative prices to have large effects. We test the model by calibrating it to 1990 data and then " backcasting " to 1961 to see what changes in crucial variables between 1961 and 1990 are predicted by the theory. The results show that, although the model is capable of explaining much of the increased concentration of trade among industrialized countries, it is not capable of explaining the enormous increase in the ratio of trade to income. Our analysis suggests that it is policy changes, rather than the elements emphasized in the new trade theory, that have been the most significant determinants of the increase in trade volume. The views expressed herein are those of the authors and not necessarily those of the Federal Reserve Bank of Minneapolis or the Federal Reserve System.