Miracle-dz/UniFAR-FTUs huggingface dataset:
The Dct−/− Mouse Model to Unravel Retinogenesis Misregulation in Patients with Albinism

We have recently identified DCT encoding dopachrome tautomerase (DCT) as the 8th gene for oculocutaneous albinism (OCA). Patients with loss of function of DCT suffer from eye hypopigmentation and retinal dystrophy. Here we investigate the eye phenotype in Dct-/- mice. We show that their retinal pigmented epithelium (RPE) is severely hypopigmented from early stages contrasting with the darker melanocytic tissues. Multimodal imaging reveals specific RPE cellular defects. Melanosomes are fewer with correct subcellular localization but disrupted melanisation. RPE cell size is globally increased and heterogeneous. P-cadherin labeling of Dct-/- newborn RPE reveals a defect in adherens junctions similar to what has been described in tyrosinase-deficient Tyrc/c embryos. The first intermediate of melanin biosynthesis, dihydroxyphenylalanine (L-Dopa), which is thought to control retinogenesis, is detected in substantial yet significantly reduced amounts in Dct-/- postnatal mouse eyecups. L-Dopa synthesis in the RPE alone remains to be evaluated during the critical period of retinogenesis. The Dct-/- mouse should prove useful in understanding the molecular regulation of retinal development and aging of the hypopigmented eye. This may guide therapeutic strategies to prevent vision deficits in patients with albinism.
A Wars2 mutant mouse shows a sex and diet specific change in fat distribution, reduced food intake and depot-specific upregulation of WAT browning

Background Increased waist-to-hip ratio (WHR) is associated with increased mortality and risk of type 2 diabetes and cardiovascular disease. The TBX15-WARS2 locus has consistently been associated with increased WHR. Previous study of the hypomorphic Wars2V117L/V117L mouse model found phenotypes including severely reduced fat mass, and white adipose tissue (WAT) browning, suggesting Wars2 could be a potential modulator of fat distribution and WAT browning. Methods To test for differences in browning induction across different adipose depots of Wars2V117L/V117L mice, we measured multiple browning markers of a 4-month old chow-fed cohort in subcutaneous and visceral WAT and brown adipose tissue (BAT). To explain previously observed fat mass loss, we also tested for the upregulation of plasma mitokines FGF21 and GDF15 and for differences in food intake in the same cohort. Finally, to test for diet-associated differences in fat distribution, we placed Wars2V117L/V117L mice on low-fat or high-fat diet (LFD, HFD) and assessed their body composition by Echo-MRI and compared terminal adipose depot weights at 6 months of age. Results The chow-fed Wars2V117L/V117L mice showed more changes in WAT browning marker gene expression in the subcutaneous inguinal WAT depot (iWAT) than in the visceral gonadal WAT depot (gWAT). These mice also demonstrated reduced food intake and elevated plasma FGF21 and GDF15, and mRNA from heart and BAT. When exposed to HFD, the Wars2V117L/V117L mice showed resistance to diet-induced obesity and a male and HFD-specific reduction of gWAT : iWAT ratio. Conclusion Severe reduction of Wars2 gene function causes a systemic phenotype which leads to upregulation of FGF21 and GDF15, resulting in reduced food intake and depot-specific changes in browning and fat mass.
A methodology to design optical systems with curved sensors

: Curved sensors are a suitable technological solution to enhance the vast majority of optical systems. In this work, we show the entire process to create curved sensor-based optical systems and the possibilities they oﬀer. This paper deﬁnes the boundaries of the reachable curvatures for a full range of monolithic sensors. We discuss how the curved focal plane shape is related to the imaged scenes and optical parameters. Two camera prototypes are designed, realized and tested, demonstrating a new compact optical architecture for a 40 degree compact objective, as well as a wide ﬁeld ﬁsheye zoom objective using a convex sensor to image a 180 degree ﬁeld of view.
Solenoid Magnet Design

D B Montgomery Chichester: John Wiley 1969 pp xii + 312 price £6 11s In his preface Dr Montgomery points out that this is a personal book, based on the design areas which have directly concerned him for the past ten years at the National Magnet Laboratory. The whole book reflects the author's own design philosophy which is based on the principle that simplicity is best.
Liquefaction-induced ground surface disruption

Although liquefaction is a major cause of earthquake damage, little harm occurs unless liquefaction generates some form of ground surface disruption or ground failure. Thus, ability to accurately predict potential for ground surface disruption is a major concern for geotechnologists charged with safe siting of constructed works. Ishihara presented preliminary empirical criteria for assessing the potential for ground surface disruption at liquefaction sites. Those criteria are based on relationships between the thickness of liquefiable sediment beneath a site and the corresponding thickness of overlying non-liquefiable soil. The purpose of this study is to further evaluate and verify these criteria by testing them against thicknesses calculated from a wide range of earthquake and site conditions. The newly developed data lead to the following conclusions: (1)For sites not susceptible to ground oscillation or lateral spread, the thickness bounds proposed by Ishihara appear to be valid. (2) For sites susceptible to ground oscillation or lateral spread, the bounds suggested by Ishihara are not sufficient for predicting ground surface disruption. (AU)
MILITANCY AND PRAGMATISM AN INTERNATIONAL PERSPECTIVE ON MARITIME LABOUR, 1870-1914*

SUMMARY: The militancy of maritime workers led worldwide to strikes of great magnitude, visibility and impact. In many countries these strikes had vast repercussions for the industrial and political development of the labour movement. As this comparative overview of maritime labour and unionism in some ten countries shows, however, after the first wave of strikes two conflicting tendencies arose which became a permanent feature of the maritime scene. The men themselves never lost their potential for militant action and adherence to radical ideologies. By contrast, many union leaders became increasingly pragmatic and even accommodationist. This article investigates the causes of this dichotomy and assesses the resulting tensions and conflicts. In many ports these led to break-away moves, spontaneous action and the replacement of moderate by radical leaders. In others the pragmatic tendency survived in power. This included a strong interest in alliances with adjacent unions and international unions.
Recombinant protein expression optimization in Escherichia coli : A review

Escherichia coli is the most extensively used organism in recombinant protein production. It has several advantages including a very short life cycle, ease of genetic manipulation and the well-known cell biology etc. which makes E. coli as the perfect host for recombinant protein expression. Despite many advantages, E. coli also have few disadvantages such as coupled transcription and translation and lack of eukaryotic post-translational modifications . These challenges can be overcome by adopting several strategies such as, using different E. coli expression vectors, changing the gene sequence without altering the functional domain, modified E. coli strain usage, changing the culture parameters and co-expression with a molecular chaperone. In this review, we present the level of strategies used to enhance the recombinant protein expression and its stability in E. coli .
Inactivation of glycogen synthase kinase-3 beta (GSK-3 beta) enhances skeletal muscle oxidative metabolism

Background: Aberrant skeletal muscle mitochondrial oxidative metabolism is a debilitating feature of chronic diseases such as chronic obstructive pulmonary disease, type 2 diabetes and chronic heart failure. Evidence in non-muscle cells suggests that glycogen synthase kinase-3 β (GSK-3 β ) represses mitochondrial biogenesis and inhibits PPAR- γ co-activator 1 (PGC-1), a master regulator of cellular oxidative metabolism. The role of GSK-3 β in the regulation of skeletal muscle oxidative metabolism is unknown. Aims: We hypothesized that inactivation of GSK-3 β stimulates muscle oxidative metabolism by activating PGC-1 signaling and explored if GSK-3 β inactivation could protect against physical inactivity-induced alterations in skeletal muscle oxidative metabolism. Methods: GSK-3 β was modulated genetically and pharmacologically in C2C12 myotubes in vitro and in skeletal muscle in vivo . Wild-type and muscle-speci ﬁ c GSK-3 β knock-out (KO) mice were subjected to hind limb suspension for 14 days. Key constituents of oxidative metabolism and PGC-1 signaling were investigated. Results: In vitro , knock-down of GSK-3 β increased mitochondrial DNA copy number, protein and mRNA abundance of oxidative phosphorylation (OXPHOS) complexes and activity of oxidative metabolic enzymes but also enhanced protein and mRNA abundance of key PGC-1 signaling constituents. Similarly, pharmacological inhibition of GSK-3 β increased transcript and protein abundance of key constituents and regulators of mi- tochondrial energy metabolism. Furthermore, GSK-3 β KO animals were protected against unloading-induced decrements in expression levels of these constituents. Conclusion: Inactivation of GSK-3 β up-regulates skeletal muscle mitochondrial metabolism and increases expression levels of PGC-1 signaling constituents. In vivo , GSK-3 β KO protects against inactivity-induced reductions in muscle metabolic gene expression.
Modelling of Dielectric Barrier Discharge Plasma Actuators for Direct Numerical Simulations

In recent years the development of devices known as plasma actuators has advanced the promise of controlling flows in new ways that increase lift, reduce drag and improve aerodynamic efficiencies; advances that may lead to safer, more efficient and quieter aircraft. The large number of parameters (location of the actuator, orientation, size, relative placement of the embedded and exposed electrodes, materials, applied voltage, frequency) affecting the performance of plasma actuators makes their development, testing and optimisation a very complicated task. Several approaches have been proposed for developing numerical models for plasma actuators. The discharge can be modelled by physics-based kinetic methods based on first principles, by semi-empirical phenomenological approaches and by PIV-based methods where the discharge is replaced by a steady-state body force. The latter approach receives a recent interest for its easy implementation in RANS and U-RANS solvers. Here, a forcing term extracted from experiments is implemented into our high-order Navier-Stokes solver (DNS) in order to evaluate its robustness and ability to mimic the effects of a surface dielectric barrier discharge. This experimental forcing term is compared to the numerical forcing term developed by Suzen & Huang (1, 2) with an emphasis on the importance of the wall-normal component of each model.
On the development of speech technology

According to the methodology of signal processing the history of speech signal processing was divided into three stages: waveform processing, parameter processing, and information processing. Both source-filter model and Hidden Markov Model have constructed the theoretical bases and supplied the powerful tools. However the products of man-machine speech communication systems based on current speech technology are still not satisfactory in practical applications. Some limitations of these models and troubles in using them were discussed in this paper. It is emphasized that the diagnostic tests have to be added to the performance evaluation of speech recognition systems and speech synthesis systems, in order to improve the operation of these models and to develop new models. The theory of "more data is better data" that seems like force-fed duck style education can not be the way out. And now the speech technology has come to a standstill, more and more fundamental researches are needed to achieve a new stage of speech technology. The real motive power of development of speech technology comes from both market pull and science and technology push.
Analysis of Security Frameworks for Mobile Cloud

The concept of Mobile Cloud Computing is relatively new in the research. It brings various advantages for mobile devices since it enables the use of Cloud resources and services. This paper gives a survey of MCC security frameworks, which helps general readers have an overview of the MCC. The issues, existing solutions, and approaches are presented. The paper is organized as follows. In the next section, we will go through some papers and get a brief idea about MCC, which helps general readers have an overview of the MCC. The issues, existing solutions, and what is actually going on in the current cloud based mobile security research and analyze two frameworks for security of mobile cloud computing.
Design of 3D Data Acquisition System on Handicrafts

The 3D data acquisition system is a very effective means to protect handicrafts. This paper analyzed the advantage of 3D laser scanning technology and designed a 3D data acquisition system on handicrafts base on it. The hardware of this system includes 6 parts: laser range finder, computer, servo system, interface board, distance data instrument, angle data instrument. The software of this system have 4 functions: parameter setting, automatic scanning control, scanning data processing, fault diagnosis. This paper discussed the operation of the software platform and development tools, modular design of software, design of software system framework and information fusion of 3D data acquisition. This paper completed the fusion and matching of point cloud data.
Aero-acoustic sources localization and high resolution imaging

Localization of Coherently Distributed (CD) source presents a challenge in the array signal processing. Our work motivates the localization of aero-acoustic source based on its spatial extension. This challenge is practically ignored in the literature of acoustic imaging field where many applications consist in mapping noisy source to reduce its contribution. The thesis presents the three following contributions. First, we propose a Joint Angle, Distance, Spread and Shape Estimator called JADSSE. The estimation of the so-called spread shape distribution parameter proposed by JADSSE avoids the modeling error due to the required a priori knowledge on the source shape when using classical estimators. Second, we expand the Decoupled DSPE to the near field. This method decouples the Direction of Arrival (DoA) and the range estimation from the spread estimation. Meanwhile, this method prevents the spread estimation for unknown shape distribution. Therefore, we propose the DADSSE to successively estimate the DOA, the range and then the spread and the shape distribution of the source. Third, we generalize the CD model and the JADSSE to consider the bi-dimensional spread of the source. Next, we propose two source power estimation approaches accounting the spatial spread of the source. The proposed methods are tested using a set of experimental data of the Renault wind tunnel application. Results show the presence of new aero-acoustic sources especially the overlapped ones with weak powers. We provide a tool to better map and characterize the aero-acoustic source by estimating the position, spread, power and shape.
Protein Kinase C as the Receptor for the Phorbol Ester Tumor Promoters: Sixth Rhoads Memorial Award Lecture1

The focus of my research has been on understanding the initial events in phorbol ester action. The phorbol esters are natural products derived from Croton tiglium, the source of croton oil, and from other plants of the family Euphorbiaceae (1). The phorbol esters initially became the object of intense research interest on the basis of their potent activity as mouse skin tumor promoters (2). application carcinogen, initiator, the action of the tumor promoters is reversible. if the chronic tumor promoter treatments precede rather than follow the treatment with the initiator, tumors do not arise.
AGGREGATE DEPENDENT RISKS – RISK MEASURE CALCULATION 1

We investigate the sum of dependent random variables. The dependent structure is modeled by copulas. The risk measures, VaR and ES of such sums, are calculated. We present the lower and upper border of VaR. The examples when the marginals have exponential and Pareto distribution are investigated. The influence of the degree of dependence on the value of VaR of the sum of dependent random variables is analysed.
Major Histocompatibility Complex Class I Molecules Bind Natural Peptide Ligands Lacking the Amino-terminal Binding Residue in Vivo *

Major histocompatibility complex (MHC) class I-pep-tide complexes are stabilized by multiple interactions, including those of the peptidic NH 2 -terminal group in the A pocket of the MHC molecule. In this study, the characterization of four natural HLA-B39 ligands lacking the amino-terminal binding residue is reported. These peptides were found in the endogenous peptide pool of one or more of the B*3901, B*3905, and B*3909 allotypes and sequenced by nanoelectrospray mass spectrometry. Control experiments ruled out that they resulted from exopeptidase trimming of their NH 2 -ter- minally extended counterparts: NAc-SHVAVENAL, EHGPNPIL, IHEPEPHIL, and EHAGVISVL, also present in the same peptide pools, during purification. HAGVISVL and HVAVENAL behaved similarly to the corresponding NH 2 -terminally extended peptides in their binding to B*3901 and B*3909 at the cell surface in vitro , and in cell surface stabilization of B*3901. This is, to our knowledge, the first demonstration that peptides lacking the amino-terminal binding residue bind in vivo to classical MHC class I molecules. The results indicate that canonical MHC-peptide interactions in the A pocket are not always necessary for endogenous peptide presentation. Both transfectants (cid:2) 2m. C1R cells grown in Dulbecco’s modified Eagle’s medium, 7.4, 7.5% heat-inactivated FBS, 100 (cid:3) g/ml streptomycin sulfate, and 100 units/ml penicillin G. RMA-S transfectant cell lines were grown in RPMI 1640 medium containing 25 m M Hepes buffer and 7.5% heat-inactivated FBS, without antibiotics but with 0.3 mg/ml hygromycin B for the B*3901 transfectant. Isolation of HLA-B39-bound Peptides— Approximately 10 10 HLA-B*3901-, B*3905-, or B*3909-C1R transfectant cells were lysed in 20 m M Tris-HCl, 150 m M NaCl, 1% Nonidet P-40, pH 7.4, containing the following protease inhibitors: 10 (cid:3) g/ml leupeptin, 2 (cid:3) g/ml pepstatin A, 2 (cid:3) g/ml aprotinin, 18.5 (cid:3) g/ml iodoacetamide, 1 m M EDTA, 348 (cid:3) g/ml phenylmethanesulfonyl fluoride, and 0.02% NaN 3 . Lysates were centri- fuged at 4 °C for 10 min at 1,500 (cid:3) g , and then for 1 h at 38,000 (cid:3) g . The supernatant was filtered through a 0.45- (cid:3) m Durapore membrane filter, pre-cleared with Sepharose-ethanolamine beads, and subjected to affinity chromatography with W6/32-Sepharose. The murine W6/32 mAb is an IgG2a, specific for a monomorphic HLA-A,B,C determinant
Prognostic significance of Notch3 immunoreactivity patterns in Caucasian colon adenocarcinoma patients

Introduction Colon cancer is traditionally regarded as the most commonly diagnosed gastrointestinal malignant disease. Nevertheless, despite advances in diagnosis and novel therapeutic options, the clinical outcomes of patients are still not satisfactory. Aim To investigate the prognostic role of Notch3, an immunohistochemical study was performed on samples of colon adenocarcinoma tissues and adjacent non-pathological mucosa in Caucasian patients. Material and methods Paraffin-embedded colon adenocarcinoma samples were assessed immunohistochemically for Notch3 protein. Connections between Notch3 immunoexpression and clinicopathological factors including the 5-year overall survival (OS) were evaluated. Results The level of the Notch3 immunohistochemical reactivity was correlated with the grade of the histological differentiation (p < 0.001). A strong expression of Notch3 protein was detected more frequently in patients with G3 tumours than in patients with G1 tumours. Moreover, the significant difference was also detected between the patients with G1 tumour and those with G2. In the G2 patients a strong reactivity was described more frequently. The Kaplan-Meier survival analysis showed that the overall survival rate in the group of patients with a low expression level of Notch3 was significantly greater than that for patients with a moderate or strong level of Notch3 immunoreactivity (p < 0.001). A multivariate analysis demonstrated that the grade of tumour differentiation (p = 0.001) and Notch3 expression (p < 0.001) were independent risk factors for worse survival. Conclusions The high level of Notch3 immunoexpression was demonstrated to be associated with malignancy-related clinicopathological factors and 5-year overall survival of patients.
TXIB-EMISSION FROM FLOOR STRUCTURE AS A MARKER OF INCREASED RISK FOR SOME SPECIFIC SYMPTOMS

Two small-scale studies were conducted in order to find out any connection between VOCemissions from floor structure and symptoms of the residents. Three blocks of flats with PVCcarpet on concrete floor were studied. In all flats residents were asked to fill out a modified questionnaire based on Orebro and Tuohilampi questionnaires. Volatile organic compounds were measured by the GC/MSD system. Higher concentrations of 2,2,4-trimethyl-1,3pentanediol di-isobutyrate (TXIB) in indoor air seem to increase the risk of symptoms like nose and eye irritation, throat symptoms and “heavy head“. Health effects may be a direct effect of TXIB exposure. Alternatively TXIB may act as a marker for another PVC component. In the world TXIB has been or is widely used as plasticizer in many PVCproducts. Results of this survey indicate that these findings are of potentially major public health importance.
Introduction and overview of recreational therapy

Recreational therapy programmes have grown in popularity with adolescents who have psychological, physical, sociological, and emotional difficulties. The literature, on the other hand, has largely concentrated on the advantages of recreation programmes, rather than on the impact of recreation therapy interventions on this vulnerable group. The purpose of this review was to conduct an examination of the literature about the impact of recreation therapy programmes. The purpose of this study was to review about the efficacy and execution of recreational therapy programmes for improving mobility outcome (e.g., balance, functional performance, fall incidence). Recreation therapy has a number of advantages. It has the potential to increase physical functionality, strengthen neuronal connections related with processing activities, and open doors to inclusion. Spending time engaging in recreational activities that interest you reduces the likelihood of depression, loneliness, and frustration. Indeed, Recreation Therapy can help individuals develop a higher feeling of self-worth and accomplishment.