Miracle-dz/UniFAR-FTUs huggingface dataset:
Autosomal recessive retinitis pigmentosa caused by mutations in the MAK gene.

PURPOSE To determine the disease expression in autosomal recessive (ar) retinitis pigmentosa (RP) caused by mutations in the MAK (male germ cell-associated kinase) gene. METHODS Patients with RP and MAK gene mutations (n = 24; age, 32-77 years at first visit) were studied by ocular examination, perimetry, and optical coherence tomography (OCT). RESULTS All but one MAK patient were homozygous for an identical truncating mutation in exon 9 and had Ashkenazi Jewish heritage. The carrier frequency of this mutation among 1207 unrelated Ashkenazi control subjects was 1 in 55, making it the most common cause of heritable retinal disease in this population and MAK-associated RP the sixth most common Mendelian disease overall in this group. Visual acuities could be normal into the eighth decade of life. Kinetic fields showed early loss in the superior-temporal quadrant. With more advanced disease, superior and midperipheral function was lost, but the nasal field remained. Only a central island was present at late stages. Pigmentary retinopathy was less prominent in the superior nasal quadrant. Rod-mediated vision was abnormal but detectable in the residual field; all patients had rod>cone dysfunction. Photoreceptor layer thickness was normal centrally but decreased with eccentricity. At the stages studied, there was no evidence of photoreceptor ciliary elongation. CONCLUSIONS The patterns of disease expression in the MAK form of arRP showed some resemblance to patterns described in autosomal dominant RP, especially the form caused by RP1 mutations. The similarity in phenotypes is of interest, considering that there is experimental evidence of interaction between Mak and RP1 in the photoreceptor cilium.
Spemann organizer formation by the intracellu

Pattern series o early c key sig are lik events Kimelm (FGF) appear equator Kimelm dorsali dorsal marginal zone blastomeres, acts to induce the dorsal organizing center, known as the Spemann organizer, in the dorsal mesoderm (Gimlich and Gerhart, 1984). The Spemann organizer then generates signals that dorsalize the adjacent mesoderm, creating the dorsoventral axis (Spemann and Mangold, 1924). Several factors have been proposed to be the dorsalizing signal that induces the Spemann organizer, including members of the Wnt family, noggin, and high levels of Vg1, all of which have the ability to induce ectopic axis formation when expressed in the ventral region of the early embryo (Smith and Harland, 1991, 1992; Sokol et al., 1991; Thomsen and Melton, 1993). Although many of the signaling factors involved in dorsoventral patterning have been identified, little is known about the intracellular signaling mechanisms by which they act. FGFs act via a MAP kinase-dependent pathway, whereas activinmediated mesoderm induction occurs through a different and, as of yet, unknown pathway (Graves et al., 1994; Hartley et al., 1994 , and oride, al., 19 PI) c ugge ntrace s. In a rsal s can b sion chlor comm rly p tral axis (Warner et al., 1984; Guthrie et al., 1988). In a few cases, large parts of intracellular signaling pathways are conserved between species, even though the signals are used to accomplish different objectives (Nishida and Gotoh, 1993). The Drosophila gene wingless (wg) encodes a member of the Wnt family, which has axis-inducing properties when expressed in the Xenopus embryo (Chakrabarti et al., 1992). Several genes have been identified that potentially act downstream of wg, including zeste-white 3/shaggy (zw3/shaggy), armadillo and dishevelled (Siegfried et al., 1992, 1994; Noordermeer et al., 1994). Zw3/shaggy encodes a cytoplasmic Ser/Thr kinase whose effects are antagonized by wg signaling (Bourouis et al., 1990; Siegfried et al., 1992). The mammalian homologs of zw3/shaggy, glycogen synthase kinase-3α and β (GSK-3α and β), have recently been shown to be regulated by targets of growth factor signaling (Sutherland et al., 1993), and zw3/shaggy/GSK-3 homologs have also been identified in yeast and plants (Bianchi et al., 1993, 1994; Pay et al., 1993; Developm Printed in
Functional programming languages for AI problem solving

Abstract Many problem domains exhibit inherent parallelism, and parallel systems which capture and exploit this can be used to look for efficient solutions to AI problems. Functional programming languages are expected to be efficiently realisable on fifth generation hardware. A rational reconstruction of AI programming paradigms is used to investigate the programmability and performance of functional languages in this particular area. Three languages—Standard ML, Hope+ and Miranda—are used in the rational reconstruction, each language being used to implement three applications. Results indicate that functional programming languages have become much more useable in recent years, and have the potential to become useful tools in AI problem solving. A brief annotated bibliography of texts which covers the introduction to, theory and implementation issues of, functional programming languages, is included.
Income predictors of smoking cessation among Hispanics

Higher socioeconomic status smokers are more successful at smoking cessation. Few studies have investigated the prospective association between multiple measures of socioeconomic status and Hispanic smoking cessation. We assessed four measures to examine which predicted smoking cessation. Hispanics without debt had 18.5 times higher odds of 30-day cessation (odds ratio = 18.47, 95% confidence interval = 3.26–104.66, p < .01) and 11-fold increased odds of 7-day point prevalence abstinence (odds ratio = 11.32, 95% confidence interval = 2.45–52.24, p < .01) at 3-month follow-up. Yearly income, education, work status, money to see a doctor, and money for medications were not predictive of smoking cessation. Debt level may better measure socioeconomic inequities by capturing objective and subjective social status associated with Hispanic smoking cessation.
Kuvvet dönüştürücülerde ısıl ve mekanik işlemlerin histerisiz hatasına etkisi

Yay elemaninin yapisal ozellikleri kuvvet donusturuculerinin performans ozelliklerini etkilemektedir. Bu calisma, cok kullanilan bir yay elamani malzemesi olan AISI 4340 celigine uygulanan farkli isil ve mekanik islemler sonrasi elde edilen ic yapisal ozelliklerin kuvvet donusturucunun histerisiz performansina etkisini arastirmaya yoneliktir. Deney sonuclarina gore, histerisiz hatasinin malzemenin sertligine paralel olarak iyilestigi, ayni sertlikteki beynitik yapinin temperlenmis martenzitik yapiya gore daha iyi performans gosterdigi, cevrimsel zorlama ile sert yapida kotulestigi, yumusak yapilarda ise iyilestigi gorulmustur. On plastik sekil degisimi ile histerisiz hatasinda iyilesme, on surunme uygulanmasi ile kotulesme goruldugu belirlenmistir. Anahtar Kelimeler : AISI 4340 celigi, k uvvet donusturucu, histerisiz hatasi, termo-mekanik islemler.
Distribution of glutamic acid decarboxylase mRNA‐containing neurons in rat medulla projecting to thoracic spinal cord in relation to monoaminergic brainstem neurons

Recent neurophysiological work has suggested the existence of monosynaptic γ‐aminobutyric acidergic (GABAergic) projections from the medulla oblongata to sympathetic preganglionic neurons. The purpose of the present study was to identify the possible anatomical location of these neurons. The location of GABAergic neurons with projection to the thoracic spinal cord was studied by using in situ hybridization for both 65‐kD and 67‐kD isoforms of glutamic acid decarboxylase (GAD) mRNA (GAD‐65 and GAD‐67, respectively) combined with midthoracic spinal cord injections of the tracer Fast Blue. Tyrosine hydroxylase (TH) or tryptophan hydroxylase immunohistochemistry was combined with GAD mRNA detection and Fast Blue to determine whether any bulbospinal catecholaminergic or serotonergic cell groups in the medulla also are GABAergic. GAD‐67 and GAD‐65 mRNA‐containing neurons had similar distribution patterns in the medulla oblongata, with some areas exhibiting lighter labeling for GAD‐65 mRNA. GABAergic bulbospinal neurons were located in the caudal part of the solitary nucleus, the parasolitary nucleus, the vestibular nuclei, the ventral medial medulla, the raphe nuclei, and parapyramidal areas. TH‐immunoreactive neurons in the A1, A2, C1, and C2 areas or the area postrema did not contain either GAD‐67 or GAD‐65 mRNA. GAD mRNA‐positive bulbospinal cells were present medial to the A1 and C1 catecholaminergic cell groups, with little or no overlap. Serotonergic neurons positive for GAD mRNA were found in the parapyramidal area and just dorsal to the pyramidal tract in the raphe magnus. This population included bulbospinal neurons. In conclusion, GABAergic neurons with projections to the thoracic spinal cord exist in a restricted number of medullary nuclei from which inhibitory sympathetic control may originate. J. Comp. Neurol. 407:367–380, 1999. © 1999 Wiley‐Liss, Inc.
Comparison of four MoM formulations when solving electrostatic problems where rotational symmetry is exploited

This paper presents results for numerical analysis of three electrostatic problems by using four MoM (method of moments) formulations while exploiting the rotational symmetry of the analyzed structures. These results are mutually compared, but also compared to the results obtained by the electrostatic solver where rotational symmetry is not considered. It is observed that exploiting the rotational symmetry reduces the number of unknowns needed for the analysis.
Off-Line Multi-Font / Size Character Recognition : A Review Miss .

Character recognition has been one of the most interesting and challenging research areas in the recent years. Many researchers develop scripts based on the different approaches used for the design of character recognition system. This paper provides a survey and classification of various character recognition techniques and describes a technique for converting textual content from a paper document into machine readable form. More precisely Character recognition is process of detecting and recognizing characters from input image and converts it into ASCII or other equivalent machine editable form. There are various approaches used for the design of OCR system, but this paper provide a review of existing works in handwritten and typewritten recognition of characters from image irrespective of their position, size, and various font styles based on the different approaches such as a Template matching, Statistical approach, Structural approach and Neural Network.
Wireless sensor networks ‘future trends and latest research challenges’

Wireless Sensor networks (WSNs) have become one of the most interesting areas of research in the past few years. A WSN is composed of a number of wireless sensor nodes which form a sensor field and a sink. These large numbers of nodes, having the abilities to sense their surroundings, perform limited computation and communicate wirelessly form the WSNs. Recent advances in wireless and electronic technologies have enabled a wide range of applications of WSNs in military, traffic surveillance, target tracking, environment monitoring, healthcare monitoring, and so on. There are many new challenges that have surfaced for the designers of WSNs, in order to meet the requirements of various applications like sensed quantities, size of nodes, and nodes’ autonomy. Therefore, improvements in the current technologies and better solutions to these challenges are required. The future developments in sensor nodes must produce very powerful and cost effective devices, so that they may be used in applications like underwater acoustic sensor systems, sensing based cyber physical systems, time critical applications, cognitive sensing and spectrum management, and security and privacy management. This paper also describes the research challenges for WSNs.
Choline phosphate cytidylyltransferase‐α is a novel antigen detected by the anti‐ERCC1 antibody 8F1 with biomarker value in patients with lung and head and neck squamous cell carcinomas

The determination of in situ protein levels of ERCC1 with the 8F1 monoclonal antibody is prognostic of survival in patients with non‐small cell lung cancer (NSCLC). The authors previously demonstrated that 8F1 recognizes a second nuclear antigen. This antigen was identified and its value as a biomarker of clinical outcomes analyzed.
The resurgence of tuberculosis: is your laboratory ready?

After years of declining case rates, tuberculosis is again a major public health problem in the United States. To compound the problem, serious outbreaks involving both patients infected with the human immunodeficiency virus (HIV) and HIV-infected and non-HIV-infected health care workers have been noted in several major metropolitan areas. Cases have also increased in other population groups, including the homeless, prisoners, migrant farm workers, and immigrants. The definitive diagnosis of tuberculosis depends on the isolation and identification of the etiologic agent, Mycobacterium tuberculosis, while design of an appropriate therapeutic regimen depends on the results of antituberculosis susceptibility testing. With this information in hand, the necessary infection control procedures and contact tracings can be initiated and informed decisions can be made regarding therapy. If laboratories cannot screen specimens rapidly for acid-fast bacilli, identify isolates in a timely manner, and provide drug susceptibility data in a short period of time, patient care may suffer and infectious patients may continue the chain of transmission. The laboratory has a major role to play in breaking this chain; the importance of completing thorough evaluations of clinical specimens for mycobacteria and, if mycobacteria are present, the importance of obtaining timely species identification and susceptibility testing results cannot be overemphasized. To halt the continuing spread of tuberculosis across the United States and to control transmission within hospitals, laboratories must recognize the urgency and optimize their procedures in reporting results of acid-fast smears, cultures, and drug susceptibility tests to clinicians.
Biochemical and Biological Characterization of a Dodecameric CD4-Ig Fusion Protein

Drug toxicities associated with HAART lend urgency to the development of new anti-HIV therapies. Inhibition of viral replication at the entry stage of the viral life cycle is an attractive strategy because it prevents de novo infection. Soluble CD4 (sCD4), the first drug in this class, failed to suppress viral replication in vivo. At least three factors contributed to this failure: sCD4 demonstrated poor neutralizing activity against most primary isolates of HIV in vitro; it demonstrated an intrinsic capacity to enhance viral replication at low concentrations; and it exhibited a relatively short half-life in vivo. Many anti-gp120 monoclonal antibodies, including neutralizing monoclonal antibodies also enhance viral replication at suboptimal concentrations. Advances in our understanding of the events leading up to viral entry suggest strategies by which this activity can be diminished. We hypothesized that by constructing a sCD4-based molecule that is large, binds multiple gp120s simultaneously, and is highly avid toward gp120, we could remove its capacity to enhance viral entry. Here we describe the construction of a polymeric CD4-IgG1 fusion protein. The hydrodynamic radius of this molecule is ∼12 nm. It can bind at least 10 gp120 subunits with binding kinetics that suggest a highly avid interaction toward virion-associated envelope. This protein does not enhance viral replication at suboptimal concentrations. These observations may aid in the design of new therapeutics and vaccines.
Quantitative absorption spectroscopy of nano-objects

We report on an optical microscopy technique aimed at measuring the absolute absorption cross section of individual nanoparticles. It relies on the thermally induced variation of the refractive index of the surrounding medium subsequent to light absorption by the nanoparticle. The technique is illustrated on gold nanoparticles featuring a well-defined plasmonic resonance. Different plasmonic modes were evidenced and quantified. The simplicity and rapidity of the measurements make it possible to investigate absorption resonances of absorbing nanostructures and microstructures in a reasonable time frame. Furthermore, the experimental approach is simply based on the use of a wavefront analyzer, which is straightforward to implement on any conventional microscope.
Formation control of unmanned aerial vehicles based on the null-space

In this paper a new algorithm for formation control of autonomous flight vehicles is presented based on multiple control objectives. The strategy includes using the null space of a Jacobian matrix to achieve different control objectives. The mission is decomposed in elementary tasks and, for each one of them, command references are generated for each robot. The different commands obtained for each task are combined through a hierarchical method using the projection in the null space, to cater multiple tasks. The proposed system is scalable, whereby the control system can be easily expanded. Finally, the stability analysis of the proposed controllers is performed and simulation results are shown, to confirm the good performance of the controllers.
Why do individuals behave differently in commons dilemmas? The case of alpine farmers using common property pastures in Grindelwald, Switzerland

The sustainable use of common-pool resources depends on users’ behaviour with regards to appropriation and provision. Most knowledge about behaviour in such situations comes from experimental research. As experiments take place in confined environments, motivational drivers and actions in the field might differ. This paper analyses farmers’ use of common property pastures in Grindelwald, Switzerland. Binary logistic regression is applied to survey data to explore the effect of farmers’ attributes on livestock endowment, appropriation and provision behaviour. Furthermore, Q methodology is used to assess the impact of broader contextual variables on the sustainability of common property pastures. It is shown that the strongest associations exist between (a) socio-economic attributes and change in livestock endowment; (b) norms and appropriation behaviour; and (c) area and pay-off and provision behaviour. Relevant contextual variables are the economic value of the resource units, off-farm income opportunities, and the subsidy structure. We conclude that with increasing farm size farmers reduce the use and maintenance of common property. Additionally, we postulate that readiness to maintain a resource increases with appropriation activities and the net returns generated from appropriation.
β2-Adrenergic Receptor-Mediated HIF-1α Upregulation Mediates Blood Brain Barrier Damage in Acute Cerebral Ischemia

Disruption of the blood brain barrier (BBB) within the thrombolytic time window is an antecedent event to intracerebral hemorrhage in ischemic stroke. Our recent studies showed that 2-h cerebral ischemia induced BBB damage in non-infarcted area and secreted matrix metalloproteinase-2 (MMP-2) accounted for this disruption. However, the factors that affect MMP-2 secretion and regulate BBB damage remains unknown. Since hypoxia-inducible factor-1 alpha (HIF-1α) was discovered as a mater regulator in hypoxia, we sought to investigate the roles of HIF-1α in BBB damage as well as the factors regulating HIF-1α expression in the ischemic brain. in vivo rat middle cerebral artery occlusion (MCAO) and in vitro oxygen glucose deprivation (OGD) models were used to mimic ischemia. Pretreatment with HIF-1α inhibitor YC-1 significantly inhibited 2-h MCAO-induced BBB damage, which was accompanied by suppressed occludin degradation and vascular endothelial growth factor (VEGF) mRNA upregulation. Interestingly, β2-adrenergic receptor (β2-AR) antagonist ICI 118551 attenuated ischemia-induced BBB damage by regulating HIF-1α expression. Double immunostaining showed that HIF-1α was upregulated in ischemic neurons but not in astrocytes andendothelial cells. Of note, HIF-1α inhibition with inhibitor YC-1 or siRNA significantly prevented OGD-induced VEGF upregulation as well as the secretion of VEGF and MMP-2 in neurons. More importantly, blocking β2-AR with ICI 118551 suppressedHIF-1α upregulation in ischemic neurons and attenuated occludin degradation induced by the conditioned media of OGD-treatedneurons. Taken together, blockade of β2-AR-mediated HIF-1α upregulation mediates BBB damage during acute cerebral ischemia. These findings provide new mechanistic understanding of early BBB damage in ischemic stroke and may help reduce thrombolysis-related hemorrhagic complications.
The Six-Month Incidence of Clinically Significant Low Back Pain in the Saskatchewan Adult Population

Study Design. A population-based, longitudinal, mailed survey was conducted. Objective. To investigate the 6-month incidence and determinants of clinically significant low back pain in the Saskatchewan adult population. Summary of Background Data. Few studies have investigated the incidence of significant low back pain in general populations. When available, such studies often differ in the assessment of pain severity. This lack of consensus in measuring pain severity results in large differences in incidence rates. Methods. A questionnaire requesting information on low back pain and other health conditions was mailed to randomly chosen individuals, ages 20 to 69, residing in the province of Saskatchewan, Canada. Of the 1131 (55%) who responded at baseline, 848 had not experienced clinically significant low back pain during the past 6 months. Clinically significant low back pain was assessed using the Chronic Pain Questionnaire, a 7-item scale that measures the intensity of chronic pain and associated disability. Individuals with no clinically significant low back pain were followed up at 6 months. Results. At the follow-up assessment, 50 individuals reported clinically significant low back pain, representing a cumulative incidence of 8% (95% confidence interval, 6–10.4). In logistic regression models, marital status, rural residency, and history of back and neck pain were associated with the onset of clinically significant low back pain. Conclusions. The 6-month incidence of clinically significant low back pain is high in Saskatchewan. It is important to prevent this condition because of the high economic and social costs associated with it.
Scale Construction: Developing Reliable and Valid Measurement Instruments

The purpose of this paper is to describe the process for developing reliable and valid measurement instruments that can be used in any hospitality industry field research setting. Many instances exist in which the researcher cannot find an adequate or appropriate existing scale to measure an important construct. In these situations it is necessary to create a new scale. Failure to carefully develop a measurement instrument can result in invalid and uninterpretable data. Hence, a systematic seven-step process is outlined here to assist researchers in devising usable scales. Examples from the authors' own research are used to illustrate some of the steps in the process.