Miracle-dz/UniFAR-FTUs huggingface dataset:
Clinical and Genetic Identification of a Large Chinese Family with Autosomal Dominant Retinitis Pigmentosa

Abstract Background: Retinitis pigmentosa (RP) is a group of inherited retinal dystrophies characterized by night blindness, progressive peripheral visual field loss, and loss of central vision. Fifty-three RP pathogenic genes are responsible for RP. Pre-mRNA processing factor 31(PRPF31) gene is the third most common cause of autosomal dominant retinitis pigmentosa (adRP), and so far more than 40 mutations in PRPF31 have been detected. Purpose: To identify the underlying genetic defect in a five-generation Chinese family affected with adRP and to study the genotype-phenotype relationship of this family. Methods: Detailed clinical investigations were undertaken and peripheral blood samples were collected from 25 individuals. Microsatellite (STR) markers tightly linked to genes known to be responsible for adRP were selected for linkage analysis. Exons and adjacent splice junctions of the candidate gene were amplified and sequenced. Results: This adRP family exhibited an incomplete penetrance of the RP phenotype. In affected individuals, age of disease onset was from infancy to 4 years of age. Typical RP features were associated with this mutation. Linkage analysis identified a maximum two-point LOD score of 3.20 with D19S418, which is close to PRPF31. A mutation PRPF31: (c.358-359 del AA) was identified by linkage analysis. Conclusions: A PRPF31 mutation was identified to be responsible for adRP in a large Chinese family. Our findings expand the mutation spectrum of RP in the Chinese population.
Option Pricing Models: The Review of Literatures (2): Bronzin (1908) and Its extensions@@@ブロンジーノ・モデル(1908)とその拡張

1. はじめに ブロンジーノ(Vinzenz Bronzin (1872–1970))は、イタリ アの応用数学者である。 Bachelier(1900)に遅れること 8 年後の 1908(明治 41)年、ドイツ語で「Theorie der Prämiengeschäfte(プレミアム契約の理論)」を著した 1。 この場合「プレミアム取引」とは、Bachelier(1900)が対 象にしたオプションと同様、原資産が先物(先渡し)契約 であるヨーロピアンオプションを意味している 2。 彼は 原資産が先渡し契約である場合のヨーロピアンオプシ ョン公式を導出した。 Bronzin(1908)のオプション価格決定式の導出方法は、 Bachelier(1900)と異なるばかりでなく、それ以降のさま ざまなオプション価格決定モデルの導出方法とも全く 異なるものであった。 その特徴は、1) 原資産について 離散的な(Bachelier(1900) の連続的なブラウン運動で なく)1 期間モデルを考え、2) ヨーロピアンオプション 価格式を行使価格で微分した結果が 1-分布関数 ( ) X F x 、すなわち生存関数 ( ) X S x になることに注目し、 さらに原資産価格に特定の分布(一様、三角、指数分布、 正規分布、二項分布など)を想定し、生存関数の不定積 分によりオプション価格の閉じた解を求めた。
THAI BUSINESS CYCLE FROM MACROECONOMIC MODEL USING BVAR AND MS-BVAR METHODS

This study aims to determine the impact of important components of Thai business cycle during prosperity and depression phases. The BVAR and MS-BVAR models are used to analyze the relationship of each variable. The variables consist of population, GDP, inflation, balance of payments, government cash balance, interest rate, and exchange rate. The data correlated in this study are secondary data during 1979 to 2014 obtained from various sources including World Bank World Development Indicators and the Global Development Finance database, World Resources Institutes (WRI), and Bank of Thailand (BOT). The results of this study indicate that each variable in this model has statistical significant relationship. From the analysis, each variable has different impact on Thai business cycle during prosperity and depression phases. Keywords: Business cycle, Macroeconomic model, BVAR, MS-BVAR
Effect of Lipopolysaccharide Derived from Pantoea agglomerans on the Phagocytic Activity of Amyloid β by Primary Murine Microglial Cells.

BACKGROUND/AIM Monophosphoryl lipid A, lipopolysaccharide (LPS)-derived Toll-like receptor (TLR) 4 agonist, has been shown to be effective in the prevention of Alzheimer's disease (AD) by enhancing phagocytosis of amyloid β (Aβ) by brain microglia. Our recent study demonstrated that oral administration of LPS derived from Pantoea agglomerans (LPSp) activates peritoneal macrophages and enhances the phagocytic activity via TLR4 signaling pathway; however, the effect of LPSp on Aβ phagocytosis in microglia is still unknown. MATERIALS AND METHODS Primary microglial cells were isolated from adult mouse brain by enzymatic digestion, following myelin removal and magnetic separation of cluster of differentiation (CD) 11b. Phagocytic analysis of the primary microglia was measured by using HiLyte™ Fluor 488-conjugated Aβ1-42 RESULTS: Using our protocols, the average yield of isolated CD11b(+) cells was around 2.2×10(5) cells per brain. CD11b(+)CD45(+)CD39(+) cells were defined here as microglia. The phagocytic activity of Aβ1-42 by the isolated microglia was confirmed. LPSp (10 ng/ml) pre-treatment for 18 h significantly increased Aβ phagocytic activity. CONCLUSION The enhancement of Aβ1-42 phagocytosis by LPSp treatment in the primary mouse microglia was demonstrated for the first time.
Post-treatment effects of subantimicrobial dose doxycycline on clinical parameters and gingival crevicular fluid transforming growth factor-beta1 in severe, generalized chronic periodontitis.

OBJECTIVE Present study aimed to evaluate the effect of 3-month adjunctive subantimicrobial dose doxycycline (SDD) on clinical parameters and gingival crevicular fluid (GCF) transforming growth factor-beta1 (TGF-beta(1)) levels in chronic periodontitis patients over 12 months. METHODS Thirty-five patients with severe, generalized periodontitis participated in the present randomized, placebo-controlled study. Patients received scaling and root planing (SRP) plus 3 months adjunctive SDD or placebo. Clinical measurements and GCF sampling were performed at baseline, 3, 6, 9 and 12 months. Eleven periodontally healthy subjects served as controls for GCF TGF-beta(1) analysis. RESULTS Clinical parameters of both SDD and placebo groups significantly improved during the study (P < 0.0125). SDD group exhibited significantly higher PD reduction at deep sites (baseline PD > or =7 mm) compared with placebo group at 6 months (P < 0.05). In SDD group significantly higher percentage of deep pockets resolved (PD reduction > or =3 mm from baseline) when compared with placebo group at 6 and 9 months (73.4% versus 49.7%; 79.9% versus 50.6%, respectively, P < 0.05). PD reduction > or =4 mm for deep pockets from baseline was also greater in SDD group than placebo at 6 months (53.4% versus 36.3%, P < 0.05). GCF TGF-beta(1) levels of SDD group was significantly higher than baseline (P < 0.0125) and placebo group (P < 0.017) at 3 months. CONCLUSIONS These results ensure further data for beneficial effects of adjunctive SDD therapy in the management of severe chronic periodontitis.
Merger and Acquisitions in India and in the World: A Trend Analysis and Future Forecasting.

Corporate restructuring through Merger and Acquisitions has become a major weapon for the corporate houses throughout the world to fight with intense competition posed by Globalization. The pace of this type of activity has further got oxygen with deregulation of various types of restrictive laws by Govt. in various times. In India after 1991 after liberalization of Indian economy, vigorous reforms in Indian corporate sector has taken places which has changed the outlook to view competition and also forced the firms to consolidate their positions in their respective areas of operations through Merger and Acquisitions. Merger and Acquisitions has been considered as the most effective form of corporate restructuring than other forms like, joint venture, strategic alliance, divestment etc. Merger and acquisition (MA these are those of the early 1900s, 1920s, 1960s, 1980s, 1990s, and 2000s.The trends of mergers and acquisitions in India have changed over the years. The immediate effects of the mergers and acquisitions have also been diverse across the various sectors of the Indian economy.
Association between Tumor Necrosis Factor-α and Disease Progression in Patients with Multiple Sclerosis

Abstract Background. Cachectin, or tumor necrosis factor-α (TNF-α), is a principal mediator of the inflammatory response and may be important in the pathogenesis and progression of multiple sclerosis, an inflammatory disease of the central nervous system. Methods. In a 24-month prospective study, we used a sensitive enzyme-linked immunosorbent assay to determine levels of TNF-α in cerebrospinal fluid and serum in 32 patients with chronic progressive multiple sclerosis and in 20 with stable multiple sclerosis and 85 with other neurologic diseases. An attempt was made to relate TNF-α levels with the degree of disability of the patients with multiple sclerosis and with their neurologic deterioration during the 24 months of observation. Results. High levels of TNF-α were found in the cerebrospinal fluid of 53 percent of the patients with chronic progressive multiple sclerosis and in none of those with stable multiple sclerosis (P<0.001). TNF-α was detected in the cerebrospinal fluid of 7 percent of the contro.
Cathodoluminescence imaging for the visualization of surface plasmon modes on metallic nanostructures

We analyzed the excitation property of a localized surface plasmon resonance (LSPR) excited with the focused electron beam. The electron beam can observe the excitation characteristic of LSPR with nanometric resolution. The gold nanorod (110 nm × 280 nm) was produced by seed mediated growth method. We measured cathodoluminescence (CL) spectrum, and obtained that two peaks were observed to 510 nm and 650 nm. We also visualaized surface plasmon modes by acquisition of CL images at spectrum peak wavelength.
A new reconstituted connective tissue matrix: preparation, biochemical, structural and mechanical studies.

A fibrinogen derivative generated by thrombin was reacted with elastin to yield a new addition product or adduct between the two proteins. Addition of fibronectin, and then of collagen, did not interfere with the basic elastin-fibrinogen reaction and conferred the qualities of an artificial connective tissue to the product. Biochemical, structural and biomechanical aspects of the new matrix were studied. Aprotinin, heparin, thiomersal, and thiourea did not inhibit the main reaction; indeed, some of these ingredients improved the matrix cohesion. Scanning electron microscopy showed the genesis of a true network whose meshes were more reticulated by the addition of thiourea. Biomechanical studies, i.e., strength and elasticity showed the thiourea matrix to be the strongest. These intrinsic properties suggest the product could have biological and clinical applications.
Modularized high frequency high power 3-level neutral point clamped PEBB cell for renewable energy system

This paper presents a modularized high frequency high power 3-level neutral point clamped power electronics building block (PEBB) for renewable energy system. Three-level diode neutral point clamped (NPC) and 3-level active NPC are most popular topologies used in industrial applications based on modularized building block. However, since the single 3-level diode NPC or active NPC cell is unable to form a 3-level DC-DC chopper, the applications of PEBB cells based on these two circuits are limited where a DC-DC chopper is needed. In this paper, loss analysis for the 3-level chopper built by two 3-level diode NPC cells or active NPC cells are conducted. Switching loops in PEBB cells are identified, and double pulse tests are conducted for turn on and turn off transient of the switching loops. Based on double pulse tests, the switching losses will be quantified for different switching loops. Two 3-level bi-directional chopper circuits based on the active NPC PEBB cells are presented and compared. Then the modified PEBB cell derived from single active NPC cell is proposed for 3-level bi-directional chopper. Based on a common active NPC PEBB cell, the power converter can be built with modularized structure to achieve high frequency, high efficiency and low cost.
Structural and Molecular Basis of the Role of Starch and Sucrose in Streptococcus mutans Biofilm Development

ABSTRACT The interaction of sucrose and starch with bacterial glucosyltransferases and human salivary amylase may enhance the pathogenic potential of Streptococcus mutans within biofilms by influencing the structural organization of the extracellular matrix and modulating the expression of genes involved in exopolysaccharide synthesis and specific sugar transport and two-component systems.
Subfornical organ efferents to paraventricular nucleus utilize angiotensin as a neurotransmitter.

In this study, we have utilized electrophysiological single unit recordings to evaluate the effects of nonpeptidergic angiotensin II (ANG II) antagonists on neural responses of hypothalamic paraventricular nucleus (PVN) neurons to either electrical stimulation in subfornical organ (SFO) or direct application of ANG II. Electrical stimulation (200-400 microA; 0.1 ms) in the SFO resulted in excitatory responses in 36 of 50 PVN neurons tested. Peristimulus histogram analysis of such excitatory effects demonstrated latencies of < 30 ms and variability of response times of approximately 50 ms in 14 of these 36 neurons. In view of previous anatomic and electrophysiological studies such inputs were therefore considered to be monosynaptically mediated by direct neural inputs from the SFO. The remaining 22 cells excited by such SFO stimulation showed responses of longer latency and duration suggestive of a different underlying synaptic mechanism. Local pressure ejection of ANG II into the PVN resulted in increased neural activity in 50% (9 of 18) of the neurons tested. After systemic (3 mg/kg iv) or local (2 x 10(-2) M; 1-25 s; 2-40 psi) microinjection of the nonpeptidergic angiotensin II1 (AT1) receptor antagonist losartan, SFO excitations were attenuated in 63.9% (23 of 36) of the PVN neurons tested, such pharmacologically blocked excitatory responses being reduced by 68.3 +/- 5.2% from control stimulation effects (P < 0.001). Similar losartan-induced attenuations of both short latency (presumed monosynaptic) (50.0%) and longer latency (72.7%) responses were observed. In addition, losartan also abolished the excitatory effects of local administration of ANG II on 77.8% (7 of 9) of ANG II-sensitive neurons in PVN tested. (ABSTRACT TRUNCATED AT 250 WORDS)
Life-Cycle Earnings Curves and Safe Savings Rates

Analyses of savings rates needed for succcessful retirement rates (SSRs) typically assume constant real earnings growth throughout one’s career. However, data on the life-cycle earnings patterns of millions of U.S. workers suggest that earnings growth does not occur at a constant rate that matches inflation. Instead, earnings tend to increase at a decreasing rate during the early years of one’s career and decrease at an increasing rate in the later years. Utilizing simulations of saving and dissaving throughout the life cycle based on both historical market returns and forecasted returns, the authors examine the impact of assuming more realistic earnings growth relative to constant inflation-adjusted growth. Results indicate that failing to account for more realistic earnings curves throughout the life cycle may overstate SSRs for lower-income households while understating SSRs for higher-income households, and understate SSRs for younger households while overstating SSRs for older households. Furthermore, historical SSRs of 10% or less are found for all but the highest-income households after accounting for more realistic earnings curves and Social Security benefits—though variations in specific effects may exist based on the simulation methods utilized.
Generalized Hessians of C1, 1-Functions and Second-Order Viscosity Subjets

Given a $C^{1,1}$-function $f:U\rightarrow R$ (where $U\subset R^{n}$ open), we deal with the question of whether or not at a given point $x_{0}\in U$ there exists a local minorant $\varphi$ of $f$ of class $C^{2}$ that satisfies $\varphi(x_{0})=f(x_{0})$, $D\varphi(x_{0})=Df(x_{0})$, and $D^{2}\varphi(x_{0})\in\mathcal{H}f(x_{0})$ (the generalized Hessian of $f$ at $x_{0}$). This question is motivated by the second-order viscosity theory of the PDEs, since for nonsmooth functions, an analogous result between subgradients and first-order viscosity subjets is known to hold in every separable Asplund space. In this work we show that the aforementioned second-order result holds true whenever $\mathcal{H}f(x_{0})$ has a minimum with respect to the positive semidefinite cone (thus, in particular, in one dimension), but it fails in two dimensions even for piecewise polynomial functions. We extend this result by introducing a new notion of directional minimum of $\mathcal{H}f(x_{0})$.
Immunisation against lactic acidosis in cattle.

The present study was designed to investigate the efficacy of control of lactic acidosis by immunisation against lactic acid-producing bacteria, Streptococcus bovis and Lactobacillus. Ten steers were allocated to two treatment groups. One group was immunised with a vaccine containing S. bovis (strain Sb-5) and Lactobacillus (LB-27) cells, and the other was a non-immunised control group. The vaccine, using Freund's complete adjuvant for primary immunisation and Freund's incomplete adjuvant for boosters, was administered intramuscularly. After primary immunisation, boosters were given at 2 to 4 week intervals. Both anti- S. bovis and anti- Lactobacillus IgG levels in saliva increased significantly (P < 0.01) after the 1st booster which were lower (P < 0.05) than the IgG levels after the 2nd and 3rd boosters, but were not significantly different (P > 0.05) from the IgG levels prior to a grain challenge (after the 4th booster). There were positive correlations between the anti- S.bovis and anti- Lactobacillus IgG in serum and saliva. Compared with the control group, steers in the immunised group had higher (P < 0.05) feed intakes, lower (P < 0.05) rumen concentrations of lactate and lower numbers of S. bovis and Lactobacillus. Three of the control animals were withdrawn from the grain challenge due to their rumen pH persisting below 5.2, while only one animal in the immunised group was withdrawn. These results suggest that the risk of lactic acidosis can be reduced by immunisation against S. bovis and Lactobacillus.
Extranuclear projections of rNST neurons expressing gustatory–elicited Fos

Previous studies have demonstrated that gustatory stimulation evokes expression of the immediate–early gene, c–fos in the rostral division of the nucleus of the solitary tract (rNST) (Harrer and Travers [1996] Brain Res. 711:125–137; DiNardo and Travers [1997] J. Neurosci. 17:3826–3839; King et al. [1999] J. Neurosci. 19:3107–3121). The present investigation further defined the phenotype of those neurons by determining their projections, by using immunohistochemistry for the Fos protein and retrograde tracing with Fluoro–Gold. Tracer injections were made into the two major extranuclear targets of rNST, the parabrachial nucleus ( PBN ) and medullary reticular formation ( RF ). These structures are thought to play differential roles in higher–order discriminative and homeostatic ( PBN ) versus reflexive function ( RF ). After PBN injections, ∼18% of the Fos–like immunoreactive (FLI) neurons were double–labeled; after RF injections the proportion was 9%. Because only a minority of FLI neurons appear to project to targets outside NST, this suggests that most of these cells have local, intranuclear projections. Comparable proportions of cells were double–labeled after sucrose or quinine, consistent with roles for both tastants in higher–order and reflexive function. On the other hand, regardless of stimulus, twice as many FLI neurons projected to the PBN as to the RF . This could suggest that more FLI neurons contribute to functions mediated by the ascending pathway. However, the results of a recent study prompted a different hypothesis: Because glossopharyngeal nerve section similarly devastates quinine–induced FLI and oral rejection but leaves discriminative function unimpaired, it was proposed that FLI neurons are more important in driving oral motor behavior than discrimination (King et al. [1999] J. Neurosci. 19:3107–3121). A plausible hypothesis for reconciling this apparent discrepancy is that many FLI neurons make local projections in rNST, that in turn give rise to RF connections. J. Comp. Neurol. 427:124–138, 2000. © 2000 Wiley‐Liss, Inc.
Identification and characterization of striatal cell subtypes using in vivo intracellular recording in rats. I. Basic physiology and response to corticostriatal fiber stimulation

The electrophysiological characteristics of two subtypes of striatal neurons, identified by their distinct patterns of response to paired impulse stimulation of corticostriatal afferents, were compared using in vivo intracellular recordings in rats. As observed in previous extracellular recording studies, the majority of neurons (73%) were found to be of the Type II class, with the remaining cells exhibiting the Type I response patern. For all cells, cortical stimulation elicited 5–30 mV EPSPs at latencies ranging from 2.0–5.3 msec; Increasing the stimulating current intensity caused a progressive increase in the amplitude of the evoked EPSPs without altering their latencies, suggesting that the EPSPs are monosynaptically mediated. Both the average amplitude and duration of the evoked EPSPs at spike threshold in Type I neurons (9.8 ± 1.7 mV, 11.8 ± 2.8 msec; mean ± SEM) were significantly smaller than those of Type II cells (20.3 ± 1.4 mV, 22.7 ± 2.1 msec). Although the average latency to the onset of the EPSP was similar for both cell classes (Type I cells: 2.3 ± 0.3 msec; Type II cells: 2.2 ± 0.2 msec), the EPSPs in Type I cells reached peak amplitude and the spikes were triggered at significantly longer latencies than in the Type II cells (peak I: 11.2 ± 2.5 msec vs. II: 7.6 ± 0.7 msec; spike I: 8.0 ± 1.2 msec vs. II: 5.7 ± 0.4 msec).
A pipelined ADC design exploration methodology employing circuit-system refinement

A pipelined ADC equation-based design space exploration methodology targeting minimum power dissipation is presented. While distinct frontiers are drawn between system-level and circuit-level design phases, this paper shows the importance of a refinement step between both phases. At the system-level, all possible architectures are examined followed by behavioral validation. Using a circuit sizing tool, different circuit topologies are investigated. The refinement phase proves to be important to increase the accuracy of system-level calculations by remapping new circuit-related parameters using the achieved circuit performances. The flow was built in an open system environment where the user has the freedom to change the modeling approach at any level, introduce different equations, and relax/tighten design constraints. An 11-bit ADC design test case is given to illustrate the methodology.
A Nonlinear Controller for the Gun Turret System

Modern nonlinear servomechanism theory is adapted to cope with the hard nonlinearities inherent in the gun turret system. This is accomplished by approximating the backlash and Coulumb friction by well behaved functions, and then synthesizing a nonlinear controller based on the scheme of "feedback stabilization" and "feedforward steady state compensation". Simulations show that this controller is indeed effective in counteracting the backlash and Coulumb friction, thus leading to a great improvement on the tracking accuracy of the gun turret system.