Miracle-dz/UniFAR-FTUs huggingface dataset:
Phenotypic expression of juvenile X-linked retinoschisis in Swedish families with different mutations in the XLRS1 gene.

OBJECTIVE To describe the clinical phenotype of juvenile X-linked retinoschisis in patients with different mutations in the XLRS1 gene. METHODS Thirty patients with 7 different XLRS1 mutations were examined. The genotype was determined by molecular genetics, which identified 6 known and 1 novel mutation (exon 5, 489 G-->T). Ophthalmologic examination included full-field electroretinogram (ERG) recordings. RESULTS The fundus appearance showed marked variations between, as well as within, families with different XLRS1 mutations. The ERG demonstrated typical reduction of B-wave amplitude, with relative A-wave preservation, causing a reduced B-A ratio in all affected males. The implicit time of the 30-Hz flicker ERG was prolonged in all patients examined. In a large family with a deletion of exon 1 and the promoter region, 12 affected males showed a phenotype ranging from moderate to severe vision impairment and a broad range of ERG abnormality, suggesting that additional factors may contribute to the disease severity. CONCLUSIONS Juvenile retinoschisis shows a wide variability in the phenotype between, as well as within, families with different genotypes. The ERG findings show reduced B-A ratios of dark-adapted recordings and prolonged implicit times of 30-Hz flicker response, which provide a useful clinical marker to confirm the clinical diagnosis. CLINICAL RELEVANCE This study describes the wide variability in the phenotype in patients with juvenile retinoschisis and different mutations in the XLRS1 gene. The study emphasizes the importance of complementing the ophthalmologic examination with full-field ERG and molecular genetics in boys with visual failure of unknown etiology to determine the diagnosis early in the course of the disease. Arch Ophthalmol. 2000;118:1098-1104
Essays on Public and Labor Economics

Chapter 1 presents evidence that current economics research significantly underestimates the effects of air pollution, regardless of the outcome of interest. This bias exists even in quasiexperimental estimates and arises because popular methods used by economists, including geographic diff-in-diffs and monitor-based interpolations, are unable to account for sharp changes in exposure over short distances. To solve this problem, I use an atmospheric dispersion model to determine the effect of every polluting firm on every house in greater Los Angeles. I then estimate the effect of NOx emissions on house prices using the exogenous variation in emissions caused by the California Electricity Crisis of 2000 and a cap-and-trade program in greater Los Angeles. The estimated price response is much larger than past estimates while conventional methods are unable to detect any effect. In Chapter 2, I use these methods to explore the equity implications of the Crisis-induced pollution reduction. I also present a locational equilibrium model and derive conditions under which lower-income residents are displaced by higher-income immigrants after an arbitrary local amenity is improved. I find that rents increased significantly in improved neighborhoods, on par with house prices. Simultaneously, total population decreased, driven by a mass outmigration of low-education residents. Low home-ownership rates among low-income households suggests that emigrants were not responding to a wealth windfall but were instead made worse off by the amenity improvement. Chapter 3 considers the principal-agent problem that arises when consumers file for bankruptcy. Lawyers advise debtors on whether to file the cheaper Chapter 7 filing or the more expensive Chapter 13 filing. Bankruptcy courts that allow lawyers to charge more for Chapter 13 see a significantly larger fraction of Chapter 13 filings. This is true controlling
Improving of Antitumor Immunity and Therapeutic Efficacy of Cancer Vaccines and Adoptive Immunotherapies Using Monoclonal Antibodies

In the past two decades, immunotherapy has become a novel therapeutic modality for cancer patients. In this therapeutic modality, the immune system of patient’s body is augmented to acquire the ability of recognition and destruction of tumor/cancer cells. Cancer immunotherapy is divided into two forms: passive immunotherapy and active immunotherapy. In passive immunotherapy monoclonal antibodies or ex vivo-proliferated/activated effector immune cells, especially T cells, are used to destroy malignant cells. Active immunotherapy is performed using various approaches, such as tumor specific/associated antigens and antigen-loaded dendritic cell vaccines, to generate patient’s immune responses against tumor/cancer cells. Recently, some approaches of immunotherapy have been approved by U.S. FDA for use in patients with advanced cancers. However, anticancer efficacy of most immunotherapeutic strategies should be improved to obtain satisfactory results in cancer patients. In this paper, we discuss the application of monoclonal antibodies targeting the cell surface molecules related with the antitumor responses of effector immune cells and their beneficial effects on cancer vaccines and other anticancer therapies.
1.6 Gb/s/pin 4-PAM signaling and circuits for a multi-drop bus

A 1.6 Gb/s/pin 4-PAM multi-drop signaling system has been implemented in 0.35-/spl mu/m CMOS. The system uses current-mode single-ended signaling, with three DC references shared across six I/O pins. A high-gain windowed integrating receiver with wide common-mode range was designed in order to improve SNR when operating with the smaller input overdrive of 4-PAM. Voltage and timing margins are measured via shmoos in a two-drop bussed system.
Prevalence of musculoskeletal activity limitation and associated factors among adults in the general population in the 1998 Quebec Health Survey.

OBJECTIVE To estimate the prevalence of short term and longterm musculoskeletal activity limitation among the general adult population of the province of Quebec, and to identify which factors are associated with these limitations. METHODS We analyzed cross-sectional data from the 1998 Quebec Health Survey. Logarithmic binomial regression models were used to estimate prevalence ratios of (1) short term activity limitation related to musculoskeletal disorders (MSD); and (2) longterm activity limitation related to MSD in relation to demographic and behavioral variables and comorbidity factors. RESULTS About one-fifth of participants reporting short term activity limitation and one-third of participants reporting longterm activity limitation mentioned MSD as a cause. Multivariate analyses showed that higher age, lower income, being overweight, ill defined nervousness, and the number of traumatic events that occurred before age 18 years were significantly associated with short term and longterm activity limitation related to MSD, whereas being in the minority ethnic group was protective for both outcomes. Female sex, physical inactivity, being a former alcohol drinker, and mental disorders were also associated with longterm activity limitation. CONCLUSION MSD are a major cause of activity limitation among adults from Quebec. With the aging population and the increasing prevalence of obesity, increasing prevalence of activity limitation related to MSD is to be expected.
An Interleaver With Arbitrary Passband Width Ratio Based on Hybrid Structure of Microring and Mach–Zehnder Interferometer

In order to improve transmission efficiency of optical-fiber communication system for hybrid 10 G/40 G system presently, and hybrid 40 G/100 G system in the future, a planar lightwave circuit-based optical interleaver with arbitrary passband width ratio is proposed and investigated, which is based on hybrid structure of microring and Mach-Zehnder interferometer and offers the advantages of compactness, ruggedness, and better compatibility with other integrated optical circuits. Most importantly, the interleaver we proposed can realize the 3-dB passband width ratio between two output ports from equal to any unequal proportion just by adjusting coupling coefficients of couplers involved. The simulation results of symmetrical and asymmetrical output spectra are presented and all of them are with a channel isolation more than 45 dB and a flat-top passband. Furthermore, the effects of some key parameters on the characteristics of output spectra are also analyzed.
C-fos gene expression in rat retinal cells after focal retinal injury.

PURPOSE To examine the expression of c-fos proto-oncogene in the rat retina after focal retinal injury. METHODS A penetrating wound was made by pushing a 22-gauge hypodermic needle through the retina into the vitreous. The retinas were analyzed by in situ hybridization using single-stranded RNA probes for c-fos transcripts and by immunocytochemistry using anti-S100 protein antibody. RESULTS Thirty minutes after the penetrating wound, c-fos mRNA was expressed in the inner nuclear layer (INL) and the ganglion cell layer (GCL) of the retina. Immunocytochemistry before in situ hybridization demonstrated c-fos mRNA surrounding the S-100 protein immunoreactive cytoplasm in the middle layer of the INL. CONCLUSIONS Focal retinal injury of the rat retina induced the expression of c-fos mRNA in retinal cells. In the INL, it is suggested that the major cells that express c-fos mRNA after focal retinal injury were the Müller cells. These results suggest that c-fos may be involved in the transcription in the Müller cells after injury to the retina.
Surface imaging microscopy, an automated method for visualizing whole embryo samples in three dimensions at high resolution

Modern biology is faced with the challenge of understanding the specification, generation, and maintenance of structures ranging from cells and tissues to organs and organisms. By acquiring images directly from the block face of an embedded sample, surface imaging microscopy (SIM) generates high‐resolution volumetric images of biological specimens across all of these scales. Surface imaging microscopy expands our range of imaging tools by generating three‐dimensional reconstructions of embryo samples at high resolution and high contrast. SIM image quality is not limited by depth or the optical properties of overlying tissue, and intrinsic or extrinsic alignment markers are not required for volume reconstruction. These volumes are highly isotropic, enabling them to be virtually sectioned in any direction without loss of image quality. Surface imaging microscopy provided a more accurate three‐dimensional representation of a chick embryo than confocal microscopy of the same sample. SIM offers excellent imaging of embryos from three major vertebrate systems in developmental biology: mouse, chicken, and frog. Immediate applications of this technology are in visualizing and understanding complex morphogenetic events and in making detailed comparisons between normal and genetically modified embryos. © 2002 Wiley‐Liss, Inc.
Investigation into factors influencing roles, relationships, and referrals in integrative medicine.

INTRODUCTION Integrative medicine (IM) is a recent phenomenon within primary care practice. It is defined variously as a process of integration or convergence of complementary and alternative medicine (CAM) with mainstream medicine or as the incorporation of alternative therapies into mainstream medical practice. Little is known about the attitude of complementary medicine practitioners regarding their place within this model or the factors that influence referral between them and medical practitioners. OBJECTIVES The aim of this research was to explore practitioners' perspectives of the theory and practice of the IM model, relevant to factors influencing referral among them. DESIGN This research applied a qualitative method with semi-structured interviews to determine practitioner perspectives of factors influencing referral in the IM setting. One family practice physician (called a general practitioner [GP] in Australia), one osteopath, and one naturopath were interviewed at each of two IM clinics in regional Australia. Thematic analysis was used to identify themes and concepts. RESULTS Thematic analysis of the transcribed data allowed for an in-depth understanding of themes and concepts surrounding practitioner perceptions of IM. Predominant themes centered on the notion of interpractitioner relationships and collaborations. Insight into these relationships within IM revealed concepts of interpractitioner trust and respect. In addition, sharing a philosophy of care and a common understanding pertaining to scope of practice and area of expertise appeared to support the IM framework. These concepts and themes were determined as important factors influencing referrals between GPs, osteopathic physicians, and naturopathic practitioners in the IM clinics studied. CONCLUSION This research has highlighted the significance of interprofessional relationships and multidisciplinary referral networks as pivotal in the efficacy of the IM clinics represented in this sample. Further research is needed to define the practitioner roles and the factors influencing referrals within IM.
Iodoacetate protects hippocampal neurons against excitotoxic and oxidative injury: involvement of heat‐shock proteins and Bcl‐2

Mild metabolic stress may increase resistance of neurons in the brain to subsequent, more severe insults, as demonstrated by the ability of ischemic pre‐conditioning and dietary restriction to protect neurons in experimental models of stroke‐ and age‐related neurodegenerative disorders. In the present study we employed iodoacetic acid (IAA), an inhibitor of glyceraldehyde‐3‐phosphate dehydrogenase, to test the hypothesis that inhibition of glycolysis can protect neurons. Pre‐treatment of cultured hippocampal neurons with IAA can protect them against cell death induced by glutamate, iron and trophic factor withdrawal. Surprisingly, protection occurred with concentrations of IAA (2–200 nm) much lower than those required to inhibit glycolysis. Pre‐treatment with IAA results in suppression of oxyradical production and stabilization of mitochondrial function in neurons after exposure to oxidative insults. Levels of the stress heat‐shock proteins HSP70 and HSP90, and of the anti‐apoptotic protein Bcl‐2, were increased in neurons exposed to IAA. Our data demonstrate that IAA can stimulate cytoprotective mechanisms within neurons, and suggest the possible use of IAA and related compounds in the prevention and/or treatment of neurodegenerative conditions.
GVoS

The exponential increase of online videos greatly enriches the life of users but also brings huge numbers of near-duplicate videos (NDVs) that seriously challenge the video websites. The video websites entail NDV-related applications such as detection of copyright violation, video monitoring, video re-ranking, and video recommendation. Since these applications adopt different features and different processing procedures due to diverse scenarios, constructing separate and special-purpose systems for them incurs considerable costs on design, implementation, and maintenance. In this article, we propose a general NDV system on Storm (GVoS)—a popular distributed real-time stream processing platform—to simultaneously support a wide variety of video applications. The generality of GVoS is achieved in two aspects. First, we extract the reusable components from various applications. Second, we conduct the communication between components via a mechanism called Stream Shared Message (SSM) that contains the video-related data. Furthermore, we present an algorithm to reduce the size of SSM in order to avoid the data explosion and decrease the network latency. The experimental results demonstrate that GVoS can achieve performance almost the same as the customized systems. Meanwhile, GVoS accomplishes remarkably higher systematic versatility and efficiently facilitates the development of various NDV-related applications.
Induction of apoptosis by cyclo‐oxygenase‐2 inhibitor NS398 through a cytochrome C‐dependent pathway in esophageal cancer cells

Non‐steroidal anti‐inflammatory drugs (NSAIDs) can induce tumor cells to undergo apoptosis in vitro. They have also shown cancer‐preventive activity in vivo. The mechanism of their effects is, however, not well defined. We investigated the mechanism by which a new NSAID, NS398, induces apoptosis in esophageal cancer cell lines. NS398 decreased cell viability in 2 cyclo‐oxygenase‐2‐positive (COX‐2+) esophageal cancer cell lines but not in a COX‐2– cell line. DNA fragmentation and TUNEL assays demonstrated that NS398 induced the 2 COX‐2+ cancer cell lines to undergo apoptosis. The percentage of apoptosis induced by NS398 was associated with the level of COX‐2 expression. Further investigation showed that the cytochrome c pathway was responsible for NS398‐induced apoptosis; i.e., cytochrome c was released from mitochondria, caspase‐9 and caspase‐3 were activated and finally poly(ADP‐ribose)polymerase (PARP) was cleaved. Furthermore, the effect of NS398 was inhibited by the caspase inhibitor Z‐DEVD‐FMK and prostaglandin E2. In contrast, bcl‐2, bax, c‐myc, Fas and Fas‐ligand showed minor changes. Altogether, our data suggest that induction of apoptosis by NS398 is associated with COX‐2 expression and occurs through the cytochrome c‐dependent pathway, which sequentially activates caspase‐9 and caspase‐3 and cleaves PARP. © 2001 Wiley‐Liss, Inc.
SAR computation for multiple close stations under modified Hata-Okumura models

The aim of the paper is to draw the attention to the intricacies and uncertainties of determining the level of electromagnetic radiation people are submitted to in urban areas where several competing telecommunications providers install numerous radio transmitters. The paper analyzes in detail an example and, based on it, proposes methods for computing with reasonable confidence the interval for the SAR levels. For this purpose, we present results obtained under a modified Hata-Okumura model.
Soluble Nogo Receptor Down-regulates Expression of Neuronal Nogo-A to Enhance Axonal Regeneration*

Nogo-A, a member of the reticulon family, is present in neurons and oligodendrocytes. Nogo-A in central nervous system (CNS) myelin prevents axonal regeneration through interaction with Nogo receptor 1, but the function of Nogo-A in neurons is less known. We found that after axonal injury, Nogo-A is increased in dorsal root ganglion (DRG) neurons unable to regenerate following a dorsal root injury or a sciatic nerve ligation-cut injury and that exposure in vitro to CNS myelin dramatically enhanced neuronal Nogo-A mRNA and protein through activation of RhoA while inhibiting neurite growth. Knocking down neuronal Nogo-A by small interfering RNA results in a marked increase of neurite outgrowth. We constructed a nonreplicating herpes simplex virus vector (QHNgSR) to express a truncated soluble fragment of Nogo receptor 1 (NgSR). NgSR released from QHNgSR prevented myelin inhibition of neurite extension by hippocampal and DRG neurons in vitro. NgSR prevents RhoA activation by myelin and decreases neuronal Nogo-A. Subcutaneous inoculation of QHNgSR to transduce DRG neurons resulted in improved regeneration of myelinated fibers in both the dorsal root and the spinal dorsal root entry zone, with concomitant improvement in sensory behavior. The results indicate that neuronal Nogo-A is an important intermediate in neurite growth dynamics and its expression is regulated by signals related to axonal injury and regeneration, that CNS myelin appears to activate signaling events that mimic axonal injury, and that NgSR released from QHNgSR may be used to improve recovery after injury.
The Politics of Benevolence: Revival Religion and American Voting Behavior

This book examines the phenomenal wave of revivals which swept the early nineteenth century, and shows the impact they had on several ideological movements. This study asks whether religious beliefs genuinely influence people's political positions or whether, instead, what appear to be religious motivations for political behavior are merely an outgrowth of their social positions. This book establihes the influence of religious beliefs and the revivalist movement on the political behavior of the past century.
Automatically Green: Behavioral Economics and Environmental Protection

Careful attention to choice architecture promises to open up new possibilities for environmental protection – possibilities that go well beyond, and that may be more effective than, the standard tools of economic incentives, mandates, and bans. How, for example, do consumers choose between environmentally-friendly products or services and alternatives that are potentially damaging to the environment but less expensive? The answer may well depend on the default rule. Indeed, green default rules may well be a more effective tool for altering outcomes than large economic incentives. The underlying reasons include the power of suggestion; inertia and procrastination; and loss aversion. If well-chosen, green defaults are likely to have large effects in reducing the economic and environmental harms associated with various products and activities. Such defaults may or may not be more expensive to consumers. In deciding whether to establish green defaults, choice architects should consider both consumer welfare and a wide range of other costs and benefits. Sometimes that assessment will argue strongly in favor of green defaults, particularly when both economic and environmental considerations point in their direction. But when choice architects lack relevant information, when interest-group maneuvering is a potential problem, and when externalities are not likely to be significant, active choosing, perhaps accompanied by various influences (including provision of relevant information), will usually be preferable to a green default.
Randomised comparison of combination chemotherapy plus supportive care with supportive care alone in patients with metastatic colorectal cancer.

OBJECTIVES--To compare the length of survival and quality of life in patients given combination chemotherapy in addition to supportive care and in patients given only supportive care. DESIGN--Randomised study. SETTING--Gastrointestinal oncology departments. PATIENTS--40 previously untreated patients with histologically confirmed, measurable colorectal cancer that was locally recurrent or metastatic. INTERVENTIONS--Patients were allocated randomly to receive chemotherapy or only supportive care in a ratio of 2:1 according to performance status, metastatic disease of the liver, and weight loss in the six months before entering the study. Chemotherapy consisted of four week cycles of intravenous leucovorin (200 mg/m2/day) followed by 5-fluorouracil (550 mg/m2/day) and cisplatin (20 mg/m2/day), each drug being given on the first four days of the cycle. MAIN OUTCOME MEASURES--Length of survival and quality of life score with an optimised functional living index-cancer scale. RESULTS--Overall survival was significantly longer for patients given chemotherapy (11.0 months) than for those receiving supportive care alone (5.0 months; p = 0.006). Despite common association of chemotherapy with mild to moderate gastrointestinal symptoms, there was no significant difference between the two groups in global or subgroup quality of life scores. In patients with abnormal scores before treatment, quality of life seemed better in the chemotherapy arm. CONCLUSIONS--In this sample of patients with disseminated colorectal cancer the chemotherapy regimen was an effective form of palliative treatment.