cssi/SciSciGPT-SciSciNet huggingface dataset:
Infantile neuronal ceroid lipofuscinosis:no longer just a ‘Finnish’ disease

The neuronal ceroid lipofuscinoses (NCLs) are a group of enigmatic neurodegenerative disorders of children that have in common the storage of autofluorescent lipofuscin, or aging pigment, in the brain. With the identification of the three major genes involved in the disorder, the NCLs are now appreciated to represent true lysosomal storage disorders. The most severe (infantile) form of NCL is caused by mutations in a lysosomal thioesterase that removes fatty acids from modified cysteine residues in proteins. Although the disorder was first described in Finland (and the identification of the underlying gene (CLN1) made in this population) defects in CLN1 and the underlying deficiency have been widely reported outside of Scandinavia. In this report, we summarize the relationship of genotype to phenotype in the disorder and evaluate known mutations in light of the recently solved crystal structure of defective enzyme, palmitoyl-protein thioesterase (PPT). We also discuss progress in identifying the fatty acyl cysteine thioesters that accumulate in PPT deficiency and in working toward animal models of NCL. Recent progress in these areas holds promise for the eventual treatment of the disorder.
Preventing enamel decalcification during orthodontic treatment

Abstract One hundred thirty-two extracted premolar teeth were selected and divided into four equal groups. The first group of thirty-three teeth received a topical application of acidulated phosphate fluoride; the second group, a topical application of stannous fluoride; the third group, an application of a polymeric adhesive coating; the fourth group was left untreated to serve as a control. On each tooth a loosely fitted orthodontic band was cemented to place. After cementation, the band was broken to simulate a loose orthodontic band in vivo. The four groups of teeth were simultaneously immersed in a decalcifying gelatin and were left undisturbed for 11 weeks. The teeth were then removed and a record was made of the decalcification produced. A chi-square test was used to compare the teeth in each group to those in every other group. Compared to a control group of teeth, those teeth treated with polymeric adhesive coating, acidulated phosphate fluoride, or stannous fluoride produced a statistically significant reduction in decalcification of the tooth surfaces beneath loose orthodontic bands. For a one-application technique, the polymeric adhesive coating provided more protection against decalcification of teeth under loose orthodontic bands than did either acidulated phosphate fluoride or stannous fluoride.
Ubiquitin-Proteasome Pathway, Alcohol and Hepatitis C Infection (HCV)

About 70% of HCV-infected patients develop chronic hepatitis with significant progression to fibrosis, cirrhosis and hepatocellular carcinoma. Numerous attempts to create an efficient vaccine for the prevention of HCV-infection failed due to extremely high mutagenesis of HCV. However, recently, sufficient progress in the treatment of HCV-infection has been achieved with appearance of highly effective direct HCV antivirals as Sofosbuvir and Ledipasvir on the market [1,2]. Multiple clinical trials are in progress to demonstrate the efficiency of these new medications alone or in a combination with already known ribavirin and interferon alpha. However “the honeymoon period” with the successful treatment by direct antivirals may not be applied to HCV patients that drink alcohol since the pathogenesis of liver disease progression in these patients is different than in alcohol non-consuming HCV patients and requires the involvement of additional therapeutic strategies.
Unique hue loci: induced shifts with complementary surrounds.

Abstract Yellow and blue unique hue loci were determined on four human observers in a neutral adaptation condition. The unique hue loci were then redetermined in a simultaneous chromatic contrast situation with the opposite unique hue used as surround. The blue loci measured in the complementary surround situation were shifted toward longer wavelengths from the loci determined with no surround. The yellow loci were shifted toward shorter wavelengths. The magnitude of the shifts tended to increase as the surround to center luminance ratio increased.
Analysis of phenylethylamines in biological tissues by gas-liquid chromatography with electron-capture detection.

Abstract 2,4-Dinitrophenyl derivatives of phenylethylamines have been applied to the sensitive quantitative analysis of phenylethylamines in biological tissues by gas chromatography and electron-capture detection. The minimal detectable amounts are about 0.1 ng. A rapid and specific cleanup procedure allows the injection of 10% of all the amines extracted from 200 mg tissues, so that as little as 20 ng/gm tissue can be determined. Amines with or without hydroxyl groups can be determined as the DNP-derivatives with equal sensitivity. The corresponding 3,5-dinitro-4-trifluoromethyl derivatives were also prepared and their chromatographic behavior is reported.
Increased NMDA responses and dendritic degeneration in human epileptic hippocampal neurons in slices

Abstract To investigate physiological properties of epileptogenic neurons in relation to epileptic pathology, intracellular recording and intracellular dye injection after the recording were obtained in dentate granule cells in slices prepared from excised human epileptic hippocampus in which selective cell degeneration has been documented. Markedly prolonged excitatory postsynaptic potentials (EPSPs) were recorded in 67% of the total neurons sampled during perforant path stimulation. Such EPSPs were voltage dependent and sensitive to the NMDA receptor antagonist d -2-amino-5-phosphonovaleric acid. Neurons that generated the increased N- methyl- d -aspartate (NMDA) responses were accompanied by abnormal dendritic morphology, i.e. loss of dendritic spines and development of beaded shafts. These findings suggest that an NMDA receptor-mediated toxic process that impinges specifically on dendritic components might take place in intractable epilepsy.
Restriction endonuclease mapping of Co1E2-P9 and Co1E3-CA38 plasmids

Abstract Using single and double restriction-endonuclease digestions, 16 and 17 cleavage sites have been mapped for the ColE2-P9 and ColE3-CA38 plasmids, respectively. One or more sites for AvaI, BglI, EcoRI, HincII, PvuI, PvuII, SmaI and XhoI endonucleases were found in both plasmids, two BglII sites were found only in ColE2-P9, and one KpnI site was unique to ColE3-CA38. ColE2-P9 was found to be slightly smaller than ColE3-CA38,4.4 Md compared to 4.6 Md. Eleven restriction sites are common to both plasmids in that they are identically placed relative to each other. These sites define a continuous DNA segment equal to over 60% of each plasmid. The remaining portions of the plasmids, which contain the non-homologous regions identified by Inselburg and Johns (1975) have no restriction sites in common, and differ in size by about 0.2 Md.
Effect of carcinogenic polycyclic aromatic hydrocarbons on mouse embryonic cells in culture: induction of spindle-shaped cells.

Abstract In cultured mouse embroyonic cells (MECs) treated with benzo[ a ]pyrene(B[ a ]P), there appeared unusual type of fibroblasts, spindle-shaped cells (SP cells), which were characterized by their narrow bipolar shape, long cellular processes and optically distinct cell borders. Appearance of SP cells was massive and irreversible. The amount of SP cells increased with increased with increasing concentrations of B[ a ]P, while early cytotoxicity did not. In various polycyclic aromatic hydrocarbons (PAHs) tested, only potent carcinogens {7,12-dimethylabenz[ a ]anthracene (DMBA), 3-methylcholanthrene (MCA), B[ a ]P and dibenz[ a , e ]pyrene (DB[ a , e ]P)} induced SP cells. Among them, PAH having higher Iball's index induced SP cells at lower concentration and at an earlier time. Weak or non-carcinogenic PAHs including 3-hydroxybenzo-[ a ]-pyrene(3-H-B[ a ]P) did not induce SP cells. α-Napthoflavon (αNF) suppressed the induction of SP cell by carcinogenic PAH. SP cells did not appear spontaneously under various abnormal culture conditions. These results indicate that carcinogenic PAHs induce the appearance of a specific type of fibroblast, SP cells in MEC cultures in accordance with their carcinogenicity.
Mechanical behavior of porous magnesium/alumina composites with high strength and low density

Abstract Porous alumina-reinforced magnesium composites were synthesized through a powder metallurgical method and characterized using optical microscope, scanning electron microscope, and compression testing. The microstructural study exhibited that the average pore sizes increased with the increase of porosity and were about 25 μm, 70 μm, and 100 μm for the samples with 10%, 28%, and 38% porosities respectively. The mechanical characterization indicated that (i) the stress–strain curves were composed of three regimes: an initial regime that deformed elastically along an approximately linear line, a long and intermediate regime, and a densification regime with a steep increase of stress; (ii) the synthesized porous magnesium composites possessed lower density and higher yield strength than those of cast dense magnesium; (iii) the average yield strength and Young's modulus were anisotropic for the porous magnesium composites synthesized in this work.
Rights, compensation, and culpability

Philosophers and laymen alike are strongly attracted to the view that the infringement of a right calls for compensation.
Historical incorporation of barium in the reef building coral Montastrea annularis at the Flower Garden Banks, north-west Gulf of Mexico

Annual growth bands of Flower Garden Banks' Montastrea annularis skeletal material were analysed for barium (Ba), a principal drilling mud component, to evaluate its potential as an indicator of water quality changes caused by oil and gas exploration and production in the northern Gulf of Mexico. The data did not indicate a Ba trend related to documented hydrocarbon exploration activities. Increased concentration variability after 1960 may, however, attest to environmental change. This variability does not correlate with a 23% decrease in average annual growth rates observed between 1957 and 1979 at the Flower Gardens.
The performance of a supercomputer built with commodity components

Abstract We built a supercomputer called Galaxy by connecting Intel Pentium-based computer nodes with Fast and Gigabit Ethernet switches. Each node has two processors at clock speeds varying from 300 to 600 MHz, up to 512 MB of memory, and small 2 Gb local disk. All nodes run the standard RedHat Linux and inter-node communication is handled by a message passing interface called MPI. Local tools are written to visualize the system performance and to balance loads. We have benchmarked a sub-Galaxy with 72 processors by NAS and Parallel LINPACK benchmark suites. We achieved 16.9 Gflops in a standard single precision LU decomposition for 46848×46848 matrix parallel LINPACK benchmark. A Galaxy with 128 processors costs approximately $250 000 and it delivers 40 Gflops of performance. This leads to a cost-performance ratio of 160 Kflops-per-dollar, which is to improve further due to increase in processor speeds and network bandwidth at similar cost. Our final system with 512 processors is expected to reach several Tflops. This article first describes the Galaxy architectural details, and then present and analyze its performance in terms of floating point number crunching, network bandwidth, and IO throughput.
Sublobar Resection Versus Definitive Radiation in Patients With Stage IA Non-Small Cell Lung Cancer

Background Many patients with resectable non-small cell lung cancer (NSCLC) are unfit for lobectomy owing to comorbidity. Surgical outcomes are biased by preoperative selection factors and upstaging that occurs during surgery. This study compares outcomes between sublobar pulmonary resection and traditional external beam radiation therapy. Methods This cohort study utilizes Surveillance, Epidemiology, and End Results–Medicare data (1998 to 2005). Patients with stage IA NSCLC treated with either radiotherapy or sublobar resection were compared. The bias of clinical staging in the radiation group versus pathologic staging in the surgical group was addressed by including only sublobar resections without lymph node sampling. Medicare claims data were used to calculate a modified Charlson comorbidity score for each patient. Results In all, 878 patients received radiotherapy and 657 underwent sublobar resection without lymph node sampling. Radiation patients were older (77.0 versus 75.5 years, p p p = 0.21). Three-year overall survival favored sublobar resection (56% versus 35%; p p p Conclusions Sublobar resection without lymph node sampling appears to be superior to radiotherapy for clinical stage IA NSCLC. For patients with prohibitive risk for lobectomy, sublobar resection may be preferable to radiotherapy. Radiotherapy results in current and future patients are likely to be better.
Age Invariance in Semantic and Episodic Metamemory: Both Younger and Older Adults Provide Accurate Feeling of Knowing For Names of Faces

Age differences in feeling-of-knowing (FOK) accuracy were examined for both episodic memory and semantic memory. Younger and older adults either viewed pictures of famous faces (semantic memory) or associated non-famous faces and names (episodic memory) and were tested on their memory for the name of the presented face. Participants viewed the faces again and made a FOK prediction about future recognition of the name associated with the presented face. Finally, four-alternative forced-choice recognition memory for the name, cued by the face, was tested and confidence judgments (CJs) were collected for each recognition response. Age differences were not obtained in semantic memory or the resolution of semantic FOKs, defined by within-person correlations of FOKs with recognition memory performance. Although age differences were obtained in level of episodic memory, there were no age differences in the resolution of episodic FOKs. FOKs for correctly recognized items correlated reliably with CJs for both types of materials, and did not differ by age group. The results indicate age invariance in monitoring of retrieval processes for name-face associations.
The Psychodynamics of Ethical behavior in Organizations

There are many aspects of organizational life—ethics prominent among them—in which things, and particularly behavior, are not what they seem. A psychodynamic approach to these apparent paradoxes helps greatly in shedding light on these areas. Much as a psycho-dynamic approach to organizational analysis has helped us see many aspects of organizational life that were less visible, so too can this approach aid us in understanding what fosters ethical behavior in organizations—and also what does not. Thus, in this article, the authors seek to develop a psychodynamic understanding of ethical behavior in organizations.
Electrochromic Shift of Chlorophyll Absorption in Photosystem I from Synechocystis sp. PCC 6803: A Probe of Optical and Dielectric Properties around the Secondary Electron Acceptor

Nanosecond absorption dynamics at approximately 685 nm after excitation of photosystem I (PS I) from Synechocystis sp. PCC 6803 is consistent with electrochromic shift of absorption bands of the Chl a pigments in the vicinity of the secondary electron acceptor A(1). Based on experimental optical data and structure-based simulations, the effective local dielectric constant has been estimated to be between 3 and 20, which suggests that electron transfer in PS I is accompanied by considerable protein relaxation. Similar effective dielectric constant values have been previously observed for the bacterial photosynthetic reaction center and indicate that protein reorganization leading to effective charge screening may be a necessary structural property of proteins that facilitate the charge transfer function. The data presented here also argue against attributing redmost absorption in PS I to closely spaced antenna chlorophylls (Chls) A38 and A39, and suggest that optical transitions of these Chls, along with that of connecting chlorophyll (A40) lie in the range 680-695 nm.
Self-perceptions of fulfilling the foster caregiver role: A preliminary analysis

Abstract This study identified items measuring foster caregivers' perceptions of fulfilling their foster caregiver role and the factors associated with those perceptions. Data were analyzed from the Illinois Foster Caregivers Study, which interviewed a sample of 204 foster caregivers who currently had children in their care. A principal component analysis determined that responses to four questions related to the foster caregiver experience measured one construct (alpha = 72). The bivariate analysis indicated that foster caregivers' age, helpfulness of the foster child information provided at the time of placement, and adult support increased the odds of foster caregivers' perceptions of fulfilling their caregiver role. Attaining at least a high school education, higher family income, depressive symptoms, and three types of childhood maltreatment decreased the odds of their positive perceptions. In the multivariate analysis, only the odds ratios for depressive symptoms, an index of childhood maltreatment, and adult support were significant predictors of the foster caregivers' perceptions. Child welfare agency and practice implications are drawn from the analysis.
Tumor-grade hyperprolactinemia induced by multiple medications in the setting of renal failure.

ABSTRACT Objective To describe a patient with galactorrhea and severe hyperprolactinemia in whom workup revealed a nontumoral mechanism. Methods We present the medical history of a woman with long-standing diabetes in whom bilateral galactorrhea and hyperprolactinemia developed. In addition, the details of her clinical course and management are reviewed. Results A 33-year-old woman with diabetes, end-stage renal disease, and gastroparesis was admitted to the hospital because of intractable nausea and vomiting. Several months before admission, she had been noted to have galactorrhea and irregular menses. Routine medications included captopril, verapamil, furosemide, prochlor-perazine, metoclopramide, cisapride, and Ortho-Novum. Laboratory evaluation showed normal thyroid function, increased serum prolactin levels (up to 1,197 ng/mL), and normal findings on magnetic resonance imaging of the pituitary. Electrophoresis of the patient's serum on a protein A Sepharose column showed no evidence of macroprolactinemia. Orally administered medications were discontinued, and the patient was given total parenteral nutrition. These measures resulted in a decrease of 300 ng/mL in serum prolactin levels in 4 days. The prolactin levels eventually normalized after withdrawal of verapamil, prochlorperazine, and metoclopramide. Conclusion A modest increase in serum prolactin level often can be produced by a variety of medications, but gross hyperprolactinemia of 200 ng/mL or higher usually raises suspicion of an underlying prolactin-secreting tumor. This case report demonstrates that conventional limits for nontumoral hyperprolactinemia can be exceeded by concurrent exposure to multiple lactotropic medications in the setting of renal failure. (Endocr Pract. 1999;5:139-142)
Temperature-dependent responsivity correction for the SPIRIT III radiometer

The spatial infrared imaging telescope (SPIRIT III) radiometer is the primary instrument aboard the Midcourse Space Experiment (MSX), which was launched on April 24, 1996. The SPIRIT III radiometer observed various orbital and suborbital targets, celestial sources, zodiacal emissions, the earth’s airglow, the aurora, and other upper atmospheric phenomena until its cryogen was depleted on February 26, 1997. During its on-orbit mission, the temperature of the radiometer focal planes rose as the cryogen was depleted, which resulted in expected changes in the radiometer’s responsivity. The responsivity’s dependency on temperature was quantified from ground calibration experiments to determine correction functions. These correction functions were verified by applying them to on-orbit observations of internal stimulators. The characterization of the dependence of responsivity on focal plane temperature is presented, the temperature effects as a function of integration mode and response level are described, and the results of applying these corrections to on-orbit internal stimulator data are given.
Meeting the needs of GLB youth in residential care settings: A framework for assessing the unique needs of a vulnerable population

Summary This article explores uses for strengths‐based assessment of gay, lesbian and bisexual youth in residential care. Gay, lesbian and bisexual (GLB) youth face unique challenges such as stigma management and disclosure. In addition, GLB youth are also at increased risk for drug use, suicide and unprotected sex. Consequently, the needs of GLB youth differ upon entering residential care. Using the strengths perspective offers a variety of techniques and strategies for advancing efficacy and efficiency of assessment for GLB youth entering residential care. Applying the strengths perspective to the assessment process seeks to increase the level of empowerment experienced by the youth, enhance rapport building between client and clinician and providing an introduction into residential care that is client‐centered and client driven. The pivotal nature of assessment in serving and treating youth in residential care make it a natural target for improved service delivery to GLB youth. Because of this populati...
The effects of visual or auditory cortical lesions on specific cross-modal transfer in the rat.

Abstract The effects of discrete visual and auditory cortical lesions on specific cross-modal transfer from vision to audition (V-A), and from audition to vision (A-V), were studied in rats, using a direct vs reversal transfer paradigm. Only sham-operated rats showed significant transfer, in both V-A and A-V. Neither visual nor auditory operates showed significant transfer in either direction. The results demonstrate the importance of visual and auditory cortex to specific transfer between the two modalities.
Investigating Workload Measures in the Nuclear Domain

Research into human-system interaction, specifically focusing on workload, has intensified in the nuclear domain. Past research on workload in the Nuclear Power Plant (NPP) domain has attempted to use both subjective and physiological measures of workload, yet the sensitivity of the workload measures used in past experiments is unknown. This initial experiment will guide future research in the NPP domain by identifying whether the NASA-TLX, EEG, and ECG are sensitive to detecting workload changes in common NPP Main Control Room (MCR) tasks. Results suggest the three workload measures did not reveal expected differences between task types in the NPP MCR context.
The Nelson-Denny Reading Test as a Predictor of College Freshman Grades:

This study represents an attempt to determine the difficulty level, the internal consistency, and the usefulness of Form C of the Nelson-Denny Reading Test (NDRT) as a predictor of freshman grades at a public, midwestern university. The 2,122 entering freshmen (37.7% male) produced a high school mean grade point average (GPA) of 3.16, a college freshmen GPA of 2.63, an American College Testing Program (ACT) Composite score of 20.6 and a NDRT of 82.8--7 points above the NDRT norm group. The internal consistency coefficients (Kuder Richardson formula 21) of the NDRT were .90 for the 100-item vocabulary section, .69 for the 36-item comprehension section, and .91 for the combined score. The NDRT was correlated positively with the verbal portion of the College Board Scholastic Aptitude Test (SAT-V) (.79), the ACT Composite score (.71), high school GPA (.39), and college freshman GPA (.33), as well as with other indicators of academic ability.High school GPA was the single most valid predictor of college freshm...
Precision electroweak experiments and heavy physics: A global analysis.

The radiative corrections of heavy degenerate and nondegenerate electroweak multiplets do not decouple from low-energy experiments. Their effects can be parametrized in a general renormalization-scheme-independent way. For isospin-breaking ${\mathit{h}}_{\mathit{V}}$ and chiral-breaking ${\mathit{h}}_{\mathit{A}\mathit{Z}}$ and ${\mathit{h}}_{\mathit{A}\mathit{W}}$, derived from the Z and W self-energies, a global analysis of existing neutral-current and Z and W data yields -1.2${\mathit{h}}_{\mathit{V}}$0.9, -3.5${\mathit{h}}_{\mathit{A}\mathit{Z}}$1.0, -4.4${\mathit{h}}_{\mathit{A}\mathit{W}}$2.4 (90% C.L.). The expectations for future experiments are described, and the implications for the top-quark and Higgs-boson masses, new generations, supersymmetry, and technicolor are discussed.
Effect of Coring Methods on Shear Values Determined by Warner‐Bratzler Sheara

SUMMARY Shear-force values were determined with the Warner-Bratzler shear machine on cores obtained by two methods from steaks from longissimus dorsi (LD) and biceps femoris (BF) muscles cooked to 61 and 80°C. One-half-inch cores were cut either parallel with the muscle fibers or perpendicular to the cut surface of the steak without regard to orientation of the fibers. The LD and BF muscles did not react in the same manner to changes in coring method. Method of coring made a large difference in shear values in LD at 80°C. With one exception shear values of paired steaks from the same muscle with cores cut parallel were more closely related than shear values of cores from within the same steak cut differently.
Linguistic politeness and face-work in computer-mediated communication, Part 1: A theoretical framework

Our daily social interaction is anchored in interpersonal discourse; accordingly, the phenomenon of linguistic politeness is prevalent in daily social interaction. Such linguistic behavior underscores the fact that linguistic politeness is a critical component of human communication. Speech participants utilize linguistic politeness to avoid and reduce social friction and enhance each other's face, or public self-image, during social interaction. It is face-work that underlies the interpersonal function of language use and encompasses all verbal and nonverbal realizations that bring forth one's positive social value, namely, face. Face-work is founded in and built into dynamic social relations; these social and cultural relations and context directly affect the enactment of face-work. Analysis and a subsequent understanding of sociointerpersonal communication are critical to the fostering of successful interaction and collaboration. Linguistic politeness theory is well positioned to provide a framework for an analysis of social interaction and interpersonal variables among discourse participants inasmuch as it is applicable not only to face-to-face social interactions but also to those interactions undertaken through online communication. © 2008 Wiley Periodicals, Inc.
Controversies in Intramedullary Nailing of Femoral Shaft Fractures

Intramedullary fixation with reaming is an excellent operative procedure that has revolutionized the treatment of fractures of the femoral shaft. Instead of being confined to bed in traction, patients can be mobilized on the first postoperative day. The expected union rate is between 95% and 99%, with infrequent malunion and infection, at least for closed fractures and for grade-1 and grade-2 open fractures. Stabilization of a femoral fracture within the first twenty-four hours after the injury has been shown to reduce morbidity and mortality in multiply injured patients. However, there are still controversial issues related to intramedullary femoral fixation. The present report addresses several of these issues, including the effect of intramedullary reaming on pulmonary complications and the rate of fracture union, whether a fracture table or a flat radiolucent table should be used for nail insertion, and whether the presence of a head injury alters the treatment selection. Classically, intramedullary nailing is done with use of a fracture table. The table assists with fracture reduction by applying sustained longitudinal traction. A perineal post provides a fulcrum against which traction is applied. The design of most fracture tables allows circumferential access to the extremity for manipulation, surgical exposure, and imaging. When surgery is delayed and length has not been maintained, the mechanical advantage of a fracture table or a femoral distractor is needed to regain length. Alternatively, intramedullary nailing can be performed on a radiolucent table1-4. Traction can be applied manually or with the use of a femoral distractor, although it takes almost as long to apply the distractor as it does to perform the entire nailing procedure and manual traction is usually sufficient to regain length. Manual traction works best when nailing is done within twenty-four hours after the injury; otherwise, muscle-shortening cannot be overcome …
Contrast-matched small-angle X-ray scattering from a heavy-atom-labeled protein in structure determination: application to a lead-substituted calmodulin-peptide complex.

The information content in 1-D solution X-ray scattering profiles is generally restricted to low-resolution shape and size information that, on its own, cannot lead to unique 3-D structures of biological macromolecules comparable to all-atom models derived from X-ray crystallography or NMR spectroscopy. Here we show that contrast-matched X-ray scattering data collected on a protein incorporating specific heavy-atom labels in 65% aqueous sucrose buffer can dramatically enhance the power of conventional small- and wide-angle X-ray scattering (SAXS/WAXS) measurements. Under contrast-matching conditions the protein is effectively invisible and the main contribution to the X-ray scattering intensity arises from the heavy atoms, allowing direct extraction of pairwise distances between them. In combination with conventional aqueous SAXS/WAXS data, supplemented by NMR-derived residual dipolar couplings (RDCs) measured in a weakly aligning medium, we show that it is possible to position protein domains relative to...
The association of cyclic parenteral nutrition and decreased incidence of cholestatic liver disease in patients with gastroschisis

Abstract Purpose The aim of the study was to investigate the effect of prophylactic cycling of parenteral nutrition (PN) on PN-induced cholestasis in patients with gastroschisis. Methods Retrospective review of initial hospital admission charts for each patient with gastroschisis from 1996 to 2007 was performed. Results One hundred seven patients were analyzed (36 prophylactically cycled, 71 control). Prophylactic cycling of PN was initiated at a mean age of 23 days (range, 7-89 days). Patients were followed for a total of 4255 days with 27 developing hyperbilirubinemia (cycled, 5; continuous, 22). Time to hyperbilirubinemia was longer in the prophylactically cycled group ( P = .005). Cumulative incidence of hyperbilirubinemia at 25 and 50 days of PN exposure was 5.7% and 9.8% (cycled) vs 22.3% and 48.8% (continuous). At any given time, children in the continuous group were 4.76 times more likely to develop hyperbilirubinemia (95% confidence interval, 1.62-14.00). After adjusting for confounding factors, children in the continuous group were 2.86 times more likely to develop hyperbilirubinemia (95% confidence interval, 0.86-9.53), but the difference was not significant ( P = .088). Conclusions Prophylactic cyclic PN is associated with a decreased incidence and prolonged time to onset of hyperbilirubinemia. Other factors, however, significantly affect this relationship. Prospective randomized investigation is warranted to investigate for a possible causal relationship.
Automated echocardiographic analysis of systemic ventricular performance in hypoplastic left heart syndrome

Abstract In patients with hypoplastic left heart syndrome, the right ventricle is positioned as the systemic ventricle. Monitoring its function is important but difficult because of its unusual position and geometry. The purposes of this study were to determine the feasibility of applying automatic echocardiographic measurements to the evaluation of right ventricular function in patients with hypoplastic left heart syndrome and to compare their function with that of normal subjects. Eleven patients with hypoplastic left heart syndrome (mean age 1.6 years) were evaluated with automatic border detection. Images were adequate if greater than 85% of the endocardium was being tracked. Systolic indexes were fractional area change and peak emptying rate; diastolic indexes were peak filling rate and the proportion of filling that occurred during rapid filling, diastasis, and atrial contraction. These data were compared with those in 18 normal control subjects. Three patients had completed stage 1 (aortic reconstruction, surgical shunt, and atrial septectomy), three patients had completed stage 2 (hemi-Fontan), and five patients had completed stage 3 (Fontan). Images were adequate in all cases. Fractional area change and peak emptying rate were significantly lower in the patients with hypoplastic left heart syndrome compared with control subjects (38% ± 6% versus 61% ± 10% and 3.5 ± 0.9 versus 5.3 ± 1.3 sec −1 , respectively). Peak filling rate was significantly lower (4.1 ± 1.3 versus 6.6 ± 2.3 sec −1 ) and atrial contribution was significantly higher (18% ± 7% versus 12% ± 4%) in the patients. Automated echocardiographic analysis is feasible in assessing systemic ventricular function in children with hypoplastic left heart syndrome. These patients have abnormal systolic and diastolic function. Automatic border detection may be useful in detecting and monitoring systemic ventricular dysfunction as these patients progress through the surgical stages of repair.
Ab-initio modeling of boron and oxygen diffusion in polycrystalline HfO2 films

We present ab-initio modeling results including formation, migration, and activation energies for B and O diffusion through bulk and grain boundaries in polycrystalline HfO 2 films. Modeling results clearly indicate that B and O can penetrate through a 40 A HfO 2 film via grain boundary diffusion, but not by bulk diffusion. SIMS analysis of B concentration profiles for polysilicon/HfO 2 /Si gate stacks after different anneals showed double B peaks at the interfaces and thus confirmed the modeling prediction.
Gaseous Atomic-Beam Light Source*

This paper describes a gaseous atomic-beam light source which has produced highly monochromatic and relatively intense spectra of the rare gases, helium, argon, neon, and xenon. The theoretical considerations governing the design of such a source are discussed. Some results of preliminary measurements are given.
Image segmentation for automated dental identification

Dental features are one of few biometric identifiers that qualify for postmortem identification; therefore, creation of an Automated Dental Identification System (ADIS) with goals and objectives similar to the Automated Fingerprint Identification System (AFIS) has received increased attention. As a part of ADIS, teeth segmentation from dental radiographs films is an essential step in the identification process. In this paper, we introduce a fully automated approach for teeth segmentation with goal to extract at least one tooth from the dental radiograph film. We evaluate our approach based on theoretical and empirical basis, and we compare its performance with the performance of other approaches introduced in the literature. The results show that our approach exhibits the lowest failure rate and the highest optimality among all full automated approaches introduced in the literature.
Dual selection enhances the signaling specificity of a variant of the quorum-sensing transcriptional activator LuxR

The transcription factor LuxR activates gene expression in response to binding the signaling molecule 3-oxo-hexanoyl-homoserine lactone (3OC6HSL), an acyl-HSL with a carbonyl substituent at the third carbon of the acyl chain. We previously described a LuxR variant, LuxR-G2E, that activates gene expression by binding a broader range of acyl-HSLs, including straight-chain acyl-HSLs to which LuxR does not respond. Here, we use a dual positive-negative selection system to identify a variant of LuxR-G2E that retains the response to straight-chain acyl-HSLs, but no longer responds to 3OC6HSL. A single mutation, R67M, reduces LuxR-G2E's response to acyl-HSLs having a carbonyl substituent at the third carbon of the acyl chain. This specificity-enhancing mutation would not have been identified by positive selection alone. The dual selection system provides a rapid and reliable method for identifying LuxR variants that have or lack the desired response to a given set of acyl-HSL signals. LuxR variants with altered signaling specificities might become useful components for constructing artificial cell-cell communication systems that program population level behaviors.
The Economic Consequences of Firms' Voluntary Submission to Greater External Scrutiny

There is a significant positive market reaction to merger announcements by acquiring firms that use lower quality external auditors. One explanation for the positive performance of these firms is that their cost-of-capital is reduced as a result of their voluntary submission to the intense scrutiny inherent in the merger process. For the lower quality audit firms, I find that the cost-of-capital effect dominates the over-valuation signaling effect of a stock-for-stock merger previously documented in the literature. I also find that a cash bid conveys to the market a stronger positive signal for low quality audit clients as opposed to high quality audit clients. Because the choice of a high quality auditor is already associated with favorable private information, clients of high quality audit firms tend to benefit less from the announcement of a cash acquisition. The results are robust to controlling for the usual determinants of acquirers' abnormal returns, both acquirers and targets' sizes, and potential sample selection biases.
Neurodevelopmental outcome of fetuses referred for ventriculomegaly

Objective To characterize the delivery and postnatal neurodevelopmental outcomes of fetuses referred for ventriculomegaly (VM).
Adenine nucleotide translocase, mitochondrial stress, and degenerative cell death

Mitochondria are intracellular organelles involved in ATP synthesis, apoptosis, calcium signaling, metabolism, and the synthesis of critical metabolic cofactors. Mitochondrial dysfunction is associated with age-related degenerative diseases. How mitochondrial dysfunction causes cell degeneration is not well understood. Recent studies have shown that mutations in the adenine nucleotide translocase (Ant) cause aging-dependent degenerative cell death (DCD) in yeast, which is sequentially manifested by inner membrane stress, mitochondrial DNA (mtDNA) loss, and progressive loss of cell viability. Ant is an abundant protein primarily involved in ADP/ATP exchange across the mitochondrial inner membrane. It also mediates basal proton leak and regulates the mitochondrial permeability transition pore. Missense mutations in the human Ant1 cause several degenerative diseases which are commonly manifested by fractional mtDNA deletions. Multiple models have been proposed to explain the Ant1-induced pathogenesis. Studies from yeast have suggested that in addition to altered nucleotide transport properties, the mutant proteins cause a global stress on the inner membrane. The mutant proteins likely interfere with general mitochondrial biogenesis in a dominant-negative manner, which secondarily destabilizes mtDNA. More recent work revealed that the Ant-induced DCD is suppressed by reduced cytosolic protein synthesis. This finding suggests a proteostatic crosstalk between mitochondria and the cytosol, which may play an important role for cell survival during aging.
A distributed state monitoring service for adaptive application management

Anubis is a simple state monitoring service that supports coordinated action among distributed management agents. It uses a temporal consistency model that addresses symmetric and asymmetric network partitions. We have used Anubis to support distributed management of adaptive applications in grid and utility computing environments and our experience has shown that the abstraction and properties provided by the service simplify the task of programming distributed management behavior. We support this claim by examining three common use cases that our developers encountered, namely: resource management, lifecycle coordination, and compositional failure management.
A segment-based approach for reconcilable model transformation

Model transformation transforms a high level source model into the skeleton of a low level target model, thereafter developers continue to refine and concretize the skeleton. With the changing requirements, the source model will be consequently modified and such modification should be reflected incrementally in the refined target model. This paper presents an end-to-end segment-based reconcilable model transformation approach to identify the conflict between source model changes and target model changes and generate a new target model which accommodates all the changes if no conflict exists. The approach is experimented in three real transformation cases.
Candidate gene association analysis for a quantitative trait, using parent-offspring trios.

With the increasing availability of genetic data, many studies of quantitative traits focus on hypotheses related to candidate genes, and also gene-environment (G×E) and gene-gene (G×G) interactions. In a population-based sample, estimates and tests of candidate gene effects can be biased by ethnic confounding, also known as population stratification bias. This paper demonstrates that even a modest degree of ethnic confounding can lead to unacceptably high type I error rates for tests of genetic effects. The parent-offspring trio design is reviewed, and several forms of the quantitative transmission disequilibrium test (QTDT) are summarized. A variation of the QTDT (QTDTM) is described that is based on a linear regression model with multiple intercepts, one per parental mating type. This and other models are expanded to allow testing of G×E and G×G interactions. A method for computing required sample sizes using direct computations is described. Sample size requirements for tests of genetic main effects and G×E and G×G interactions are compared across various QTDT approaches to infer their efficiencies relative to one another. The QTDTM is found to meet or exceed the efficiency of other QTDT approaches. For example, the QTDTM is approximately 3% more efficient than the QTDT of Rabinowitz ([1997] Hum. Hered. 47:342–350) for testing a genetic main effect, but can be as much as twice as efficient for testing G×E interaction, and three times more efficient for testing G×G interaction. Genet Epidemiol 25:327–338, 2003. © 2003 Wiley-Liss, Inc.
Evaluation of Differentially Expressed Proteins Following Serum Exposure in Avian Pathogenic Escherichia coli

Colibacillosis, caused by avian pathogenic Escherichia coli (APEC), is an extraintestinal disease that causes great economic loss to the poultry industry each year. APEC must overcome host defenses, such as immune system components found in serum, in order to establish infection; however, the mechanism of such serum resistance has been elusive. In the present study, a proteomic approach was used to evaluate APEC proteins that were differentially expressed after exposure to chicken serum to identify specific proteins that may be involved in serum resistance of APEC isolates. Proteins were isolated and separated by two-dimensional (2D) gel electrophoresis, and 10 protein spots corresponding to differentially expressed proteins were chosen for sequencing using electrospray ionization tandem mass spectrometry. Eight proteins were identified among the spots, some of which have previously been associated with the virulence of E. coli. Significantly, an outer-membrane protein previously associated with serum resistance, OmpA, was among those proteins identified, further indicating that differential regulation of this protein may be involved in serum resistance. This study opens the door to future research using a proteomic approach to identify the key players in serum resistance of APEC.
Production of linolenic acid in yeast cells expressing an omega-3 desaturase from tung (Aleurites fordii).

Tung oil is an industrial drying oil containing ca. 90% PUFA. We previously reported on enzymes required for the synthesis of linoleic (6% of FA) and eleostearic (80%) acids and here describe the cloning and functional analysis of an omega-3 FA desaturase (FAD3) required for the synthesis of linolenic acid (1%). The tung FAD3 cDNA was identified by screening a tung seed cDNA library using the polymerase chain reaction and degenerate primers encoding conserved regions of the FAD3 enzyme family. Expression of this cDNA in yeast cells, cultured in the presence of linoleic acid, resulted in the synthesis and accumulation of linolenic acid, which accounted for up to 18% w/w of total cellular FA. Tung FAD3 activity was significantly affected by cultivation temperature, with the greatest amount of linolenic acid accumulating in yeast cells grown at 15°C. The amount of linolenic acid synthesized in yeast cells by tung FAD3 is ca. 10-fold higher than that observed by expression of a rapeseed (Brassica napus) FAD3 in yeast, suggesting that tung FAD3 might be useful for biotechnological production of omega-3 FA in transgenic organisms.
Interaction of an Aromatic Molecule with a Surface

The interaction between an aromatic molecule and the graphite basal plane is constructed using a Lennard-Jones 6–12 potential. The corrugation of the potential across the surface, the binding energies and vibrational frequencies of the aromatic molecules on the basal plane have been calculated and compared with experimental data. We have also calculated the interaction between a rare-gas atom and an aromatic molecule using two independent models, one based on the pairwise sum of the Lennard-Jones potential and the other on the Effective Medium Theory (EMT) for the repulsive part and a Van der Waals dispersion term for the attractive part. Our results are then compared with available experimental data.
Phagocyte NADPH oxidase, but not inducible nitric oxide synthase, is essential for early control of Burkholderia cepacia and chromobacterium violaceum infection in mice.

Chronic granulomatous disease (CGD) is an inherited disorder of the NADPH oxidase in which phagocytes are defective in generating superoxide anion and downstream microbicidal metabolites, including hydrogen peroxide, hydroxyl anion, and hypohalous acid. As a result of the defect in this key antimicrobial pathway, patients with CGD suffer from recurrent life-threatening infections by catalase-producing bacteria and fungi (4, 28). Burkholderia (Pseudomonas) cepacia (23, 32) and Chromobacterium violaceum (4, 31; A. M. Macher, T. B. Casale, J. I. Gallin, H. Boltansky, and A. S. Fauci, Letter, Ann. Intern. Med. 98:259, 1983) are highly virulent pathogens in patients with CGD. B. cepacia and other Burkholderia species typically cause pneumonia and, less commonly, sepsis in patients with CGD (3, 23, 25, 32). C. violaceum, a rare pathogen typically encountered in brackish waters, occurs with unusually high frequency in CGD patients from the southern United States (e.g., Florida and Louisiana), although C. violaceum infection in the northern United States has been reported (4, 31; Macher et al., letter). Clinical manifestations include soft tissue infection, pneumonia, and sepsis. In the present study, we compared the levels of virulence of B. cepacia and C. violaceum in p47phox−/− mice and in mice with a targeted disruption of the inducible nitric oxide synthase (iNOS) gene to assess the relative importance of the reactive oxygen and reactive nitrogen intermediate pathways in host defense against these pathogens.