Miracle-dz/UniFAR-FTUs huggingface dataset:
Longitudinal changes in cognitive and adaptive behavior in fragile X females: a prospective multicenter analysis.

In prospective studies of young, fragile X [fra(X)] males with the full mutation, cognitive abilities (IQ scores) and adaptive behavior levels (DQ scores) declined in most subjects tested. Little is known about longitudinal changes in IQ and DQ scores in young fra(X) females, although one earlier retrospective study showed declines in IQ scores in 8 of 11 subjects. To examine fra(X) females prospectively, we tested and retested 13 females with the full mutation, age 4 to 15 years. Nine were tested and retested in North America, and four were evaluated at the Catholic University in Leuven, Belgium. Cognitive abilities of North American females were measured using the Stanford-Binet 4th Edition. Adaptive behavior levels were ascertained from the Vineland Adaptive Behavior Scales. For Belgians, test-retest scores from the Wechsler Intelligence Scales for Children-Revised were used. Subjects were subsequently separated into two age cohorts: those tested initially before age 7 years and those tested initially after age 7 years. Compared with young males with the full mutation and of the same age, females expectedly display a wider range of IQ scores. Test-retest IQ scores showed statistically significant decreases (P < 0.03). Analysis of individual test-retest scores indicate that declines in eight females were statistically significant. Adaptive behavior scores were available only for North American females. Five of nine (55%) showed significant declines in DQ. Like young males with the full mutation, all females with the full mutation attained higher adaptive behavior levels than cognitive scores, i.e., DQ > IQ.
Surfactant Protein-D Regulates Surfactant Phospholipid Homeostasis in Vivo *

Surfactant protein D (SP-D) is a 43-kDa member of the collectin family of collagenous lectin domain-containing proteins that is expressed in epithelial cells of the lung. The SP-D gene was targeted by homologous recombination in embryonic stem cells that were used to produce SP-D (±) and SP-D (−/−) mice. Both SP-D (−/−) and SP-D (±) mice survived normally in the perinatal and postnatal periods. Whereas no abnormalities were observed in SP-D (±) mice, alveolar and tissue phosphatidylcholine pool sizes were markedly increased in SP-D (−/−) mice. Increased numbers of large foamy alveolar macrophages and enlarged alveoli were also observed in SP-D (−/−) mice. Phospholipid composition was unaltered in SP-D (−/−) mice, but surfactant morphology was abnormal, consisting of dense phospholipid membranous arrays with decreased tubular myelin. The pulmonary lipoidosis in the SP-D (−/−) mice was not associated with accumulation of surfactant proteins B or C, or their mRNAs, distinguishing the disorder from alveolar proteinosis syndromes. Surfactant protein A mRNA was reduced and, SP-A protein appeared to be reduced in SP-D (−/−) compared with wild type mice. Targeting of the mouse SP-D gene caused accumulation of surfactant lipid and altered phospholipid structures, demonstrating a previously unsuspected role for SP-D in surfactant lipid homeostasis in vivo.
Can cancer registries show whether treatment is contributing to survival increases for melanoma of the skin at a population level?

RATIONALE, AIMS AND OBJECTIVES It is uncertain whether survival increases from melanoma recorded by some population registries include a treatment effect. The US Surveillance, Epidemiology and End Results (SEER) programme has good data quality control, large numbers of cases enabling high statistical precision and summary stage plus thickness, which we consider to be a best-case population registry scenario to investigate potential for a treatment effect. We have investigated SEER data to indicate whether survivals increases are fully attributable to earlier diagnosis and other non-treatment factors. METHODS Through relative survival regression, the effects of diagnostic period on 5-year excess mortality were investigated, adjusting for socio-demographic factors, lesion sub-site, histology, thickness and stage at diagnosis in 1990-2009 (n = 99 690 cases). RESULTS The reduction in excess mortality (95% confidence interval) between 1990-1999 and 2000-2009 was 31 (20-41)% for localised melanoma, 18 (12-22)% for regional melanoma and 3 (-5-10)% for melanomas with distant spread. Younger age was predictive of a greater percentage reduction. Treatment benefits are inferred from the higher survivals in 2000-2009 but uncertainty remains due to incomplete data to adjust for non-treatment factors and a lack of treatment data. CONCLUSIONS Registries should use new information systems to collect more complete data on stage, other prognostic indicators, co-morbidities and treatment, to provide more definitive and detailed information on population effects of cancer control.
Selective suppression of sphincter activation during sacral anterior nerve root stimulation

The purpose of this work was to electrically activate small‐diameter motor fibers in the sacral anterior roots innervating the urinary bladder, without activating the large‐diameter fibers to the sphincter. Quasitrapezoidal current pulses were applied through tripolar spiral nerve electrodes on selected anterior sacral roots during acute experiments on eight dogs, maintained under pentobarbital anesthesia. Pressures were recorded from the bladder and sphincter with catheter‐mounted gauges. Stimulation with biphasic quasitrapezoidal pulses showed decrease in sphincter recruitment with increasing pulse amplitudes. The minimum current amplitude that resulted in maximum sphincter suppression was used to stimulate the roots with trains of 20 Hz pulses, with 60 mL of saline filling the bladder. Pressures were also recorded when 100 μs rectangular pulse trains at 20 Hz, both continuous and intermittent, were applied. Trains of stimuli were applied before and after dorsal root rhizotomy. Suppression of sphincter activation was defined to be a percentage, [(Maximum pressure −Minimum pressure)/Maximum pressure × 100. The results from 22 roots in eight animals show that with single pulses, the average percentage suppression of sphincter activation was 76.3% (± 14.0). The minimum current for maximum sphincter suppression was 1.29 mA (± 0.62). The average bladder pressure evoked was 50 cm of water during pulse train stimulation, with no significant difference due to pulse type. With pulse trains, the sphincter pressures were significantly higher when the bladder was filled. Evacuation of fluid occurred in three animals with average flow rates of 1.0 mL/s. Neurourol. Urodynam. 21:55–64, 2002. © 2002 Wiley‐Liss, Inc.
Effect of molecular therapeutics on liver regeneration in a murine model.

PURPOSE Unresectable metastatic colorectal cancer (CRC) can be rendered resectable with systemic chemotherapy in approximately 20% of cases. Most patients with metastatic CRC receive chemotherapy with the addition of targeted therapy with anti-vascular endothelial growth factor (VEGF) or anti-epidermal growth factor receptor (EGFR) antibodies. We sought to determine whether anti-VEGF receptor (VEGFR) or anti-EGFR therapy would impair liver regeneration after partial hepatectomy (PH) in mice. MATERIALS AND METHODS Mice underwent either 66% PH or sham laparotomy. In the first experiment, mice in the PH group were randomly assigned to receive daily intraperitoneal injections of monoclonal antibodies (MoABs) to murine VEGFR-2 or nonspecific MoABs (control). In the second experiment, mice in the PH group were randomly assigned to receive intraperitoneal injections of antimurine EGFR or nonspecific (control) MoABs. In both experiments, therapy was initiated the day before surgery and continued until the mice were killed on day 5. Livers were collected and processed. RESULTS Anti-VEGFR-2 therapy slightly impaired liver regeneration and hepatic cell proliferation compared with control. Hematoxylin and eosin staining showed no differences in liver morphology. CD105 staining showed decreased levels of activated endothelium in livers in the VEGFR-2 MoAB group. VEGFR-2 MoAB therapy decreased the levels of the cell cycle regulators cyclin D1 and cyclin D3 and the regenerative cytokine interleukin-6. Anti-EGFR therapy had no effect on liver regeneration or cellular proliferation. CONCLUSION Anti-VEGFR-2 therapy slightly impaired liver regeneration in this murine model, whereas anti-EGFR therapy had no effect on liver regeneration.
Impact of carbon nanotube morphology on phenanthrene adsorption

The present study examined the roles of the specific surface area (SSA), diameter, and length of carbon nanotubes (CNT) on the adsorption of phenanthrene (PNT) by analyzing the adsorption isotherms obtained with several single‐walled carbon nanotubes (SWNT) and multiwalled carbon nanotubes (MWNT). At low equilibrium concentrations (e.g., 1 ppb), MWNTs with larger outer diameters exhibited higher PNT adsorption capacity on an SSA basis than those with smaller diameters. With increasing equilibrium concentration, adsorption on an SSA basis became independent of MWNT diameter, and the total surface area controlled maximum adsorption capacity. A similar analysis for the adsorption of naphthalene, a planar molecule with one less benzene ring but 20 times higher solubility than PNT, showed no correlation with respect to MWNT outer diameter. The results indicated that the surface curvature of MWNT was more important on the adsorption of PNT than on the adsorption of naphthalene. Specific surface area normalized isotherms did not show a correlation between PNT adsorption and lengths of SWNTs and MWNTs. Characterization results indicated that the morphology of CNTs plays an important role on the SSA and pore volume. Data from the manufacturer may not always represent the characteristics of CNTs in a particular batch. Therefore, accurate characterization of CNTs is critical to systematically examine the behavior of CNTs, such as adsorption and transport, in environmental systems. Environ. Toxicol. Chem. 2012;31:73–78. © 2011 SETAC
A new world : the geopolitics of the energy transformation

Aware that the growing deployment of renewables has set in motion a global energy transformation with significant implications for geopolitics, Adnan Z. Amin, the Director-General of the International Renewable Energy Agency (IRENA), with the support of the Governments of Germany, Norway and the United Arab Emirates, convened the Global Commission in January 2018.Chaired by former President Olafur Ragnar Grimsson of Iceland, the Commission comprises a diverse group of distinguished leaders from the worlds of politics, energy, economics, trade, environment and development. The Commission is an independent body with members serving in their individual capacity.This Report analyzes the geopolitical implications of the global energy transformation driven by renewables. It is the culmination of ten months’ deliberations by the Commission, involving four meetings held in Berlin, Oslo Reykjavik and Abu Dhabi respectively, as well as consultations with business leaders, academics and policy thinkers. It is informed by a number of background papers drafted by experts in the fields of energy, security and geopolitics.The Commission takes full and independent responsibility for this Report, which reflects the consensus of its members.
Household characteristics at the bottom, the typical, and the top of the 2016 income distribution in South Korea: A quantile regression analysis

Reg Sci Policy Pract. 2019;11:5–21. Abstract This paper seeks understanding how demographic characteristics affect the different quantiles of the South Korean income distribution. Findings from the 2016 Household Income and Expenditure Survey data suggest that, at the bottom of the distribution, households relied heavily on cash transfers from institutions and other households, while those at the top relied more on wages, suggesting that high‐income households in South Korea were not members of the “propertied class. ” We then develop simple empirical models to measure the effect of demographic covariates on household income conditional on a number of variables commonly used in inequality studies, including lifecycle, education, gender, marital status, urban residence, job security, industry and occupation type. Quantile regression results demonstrate how a particular quantile of the conditional distribution changes with demographic characteristics. Specifically, we found an increase in gender and unemployment effects as we go from high to low quantiles. Programmes aimed at combating gender discrimination and persistent unemployment therefore are expected to be inequality reducing. This is in contrast to policies to promote higher education or to expand public sector employment that quantile regressionmodels indicate to be inequality enhancing.
Time representation of mitochondrial morphology and function after acute spinal cord injury

Changes in mitochondrial morphology and function play an important role in secondary damage after acute spinal cord injury. We recorded the time representation of mitochondrial morphology and function in rats with acute spinal cord injury. Results showed that mitochondria had an irregular shape, and increased in size. Mitochondrial cristae were disordered and mitochondrial membrane rupture was visible at 2-24 hours after injury. Fusion protein mitofusin 1 expression gradually increased, peaked at 8 hours after injury, and then decreased to its lowest level at 24 hours. Expression of dynamin-related protein 1, amitochondrial fission protein, showed the opposite kinetics. At 2-24 hours after acute spinal cord injury, malondialdehyde content, cytochrome c levels and caspase-3 expression were increased, but glutathione content, adenosine triphosphate content, Na + -K + -ATPase activity and mitochondrial membrane potential were gradually reduced. Furthermore, mitochondrial morphology altered during the acute stage of spinal cord injury. Fusion was important within the first 8 hours, but fission played a key role at 24 hours. Oxidative stress was inhibited, biological productivity was diminished, and mitochondrial membrane potential and permeability were reduced in the acute stage of injury. In summary, mitochondrial apoptosis is activated when the time of spinal cord injury is prolonged.
A holistic perspective of patients' lives post‐Ilizarov external fixation

The Ilizarov external fixator (IEF) is used in the treatment of complicated fractures with severe soft tissue damage. Despite advantages of being minimally invasive and allowing early weight‐bearing, the IEF has limitations, including size, treatment duration and crucial pin‐site care. Many patients showed enormous resilience despite the devastating effects of their injuries. Our aim was to assess the value of IEF treatment for trauma patients with a focus on their quality of life.
The Degree of Oligomerization of the H-NS Nucleoid Structuring Protein Is Related to Specific Binding to DNA*

At several E. coli promoters, initiation of transcription is repressed by a tight nucleoprotein complex formed by the assembly of the H-NS protein. In order to characterize the relationship between the structure of H-NS oligomers in solution and on relevant DNA fragments, we have compared wild-type H-NS and several transdominant H-NS mutants using gel shift assays, DNase I footprinting, analytical ultracentrifugation, and reactivity toward a cross-linking reagent. In solution, oligomerization occurs through two protein interfaces, one necessary to construct a dimeric core (and involving residues 1–64) and the other required for subsequent assembly of these dimers. We show that, as well as region 64–95, residues present in the NH2-terminal coiled coil domain also participate in this second interface. Our results support the view that the same interacting interfaces are also involved on the DNA. We propose that the dimeric core recognizes specific motifs, with the second interface being critical for their correct head to tail assembly. The COOH-terminal domain of the protein contains the DNA binding motif essential for the discrimination of this specific functional assembly over competitive nonspecific H-NS polymers.
MRI changes in psoriatic dactylitis--extent of pathology, relationship to tenderness and correlation with clinical indices.

OBJECTIVES To quantify the extent of inflammation in psoriatic dactylitis and to examine the relationship between clinical and magnetic resonance imaging (MRI) data in both tender and non-tender dactylitis. METHODS Seventeen patients with psoriatic dactylitis underwent clinical assessment for 6 months after change of treatment, usually to methotrexate. Measures of dactylitis included the Leeds Dactylitis Index, the assessment tool used in the Infliximab in Psoriatic Arthritis Clinical Trial (IMPACT), a simple count of tender dactlylitic digits and a count of all dactylitic digits, both tender and non-tender. MRI scans of the affected hand or foot were performed before and after treatment using a 1.5T Siemen's scanner pre- and post-contrast. RESULTS All patients improved clinically, as did their respective dactylitis scores and MRI images. The findings on MRI in both dactylitic and non-dactylitic digits were profound and widespread. The difference between tender and non-tender dactylitis was quantitative rather than qualitative. Synovitis and soft-tissue oedema were the most frequent abnormalities being present in 69% of tender dactylitic digits but bone oedema and flexor tenosynovitis were also frequently seen. Soft-tissue oedema was circumferential and enhancing and not limited to association with the flexor or extensor tendons. None of the clinical indices of dactylitis showed a close relationship to the extent of MRI abnormalities. CONCLUSIONS MRI images demonstrate widespread abnormalities in digits of people with psoriatic arthritis. Tender dactylitic digits have more abnormalities than other digits but the relationship between clinical and MRI scores is not strong.
Destruction of Place: Policy Diffusion and Schenectady's Urban Redevelopment

II TABLE OF CONTENTS III CHAPTER ONE: POLICY DIFFUSION AND URBAN REDEVELOPMENT STRATEGY 4 Section One: What is Policy Diffusion? 4 Section Two: Jane Jacobs and Busy Streets 8 Section Three: Types of Urban Redevelopers 12 Section Four: Import Replacement in Urban Redevelopment 14 Section Five: Courting the Creative Class 17 DESCRIPTION OF THE MODEL 23 CHAPTER TWO: POLICY DIFFUSION AND SCHENECTADY’S REDEVELOPMENT 25 Section One: Schenectady’s Early Planning 25 Section Two: A History of Federal Redevelopment Programs 26 Section Three: Urban Renewal In Schenectady 32 Section Four: Background on City Decision-Making 35 Section Five: Later Redevelopment of the Federal Era 38 CHAPTER 3: METROPLEX AND NON-DIFFUSED REDEVELOPMENT 45 Section One: Creation and Contrast 45 Section Two: Three Examples of Projects 49 Section Three: Lessons on Redevelopment 56
Linker scanning mutagenesis of the internal ribosome entry site of poliovirus RNA

The initiation of cap-independent translation of poliovirus mRNA occurs as a result of ribosome entry at an internal site(s) within the 5' noncoding region. A series of linker scanning mutations was constructed to define the genetic determinants of RNA-protein interactions that lead to high-fidelity translation of this unusual viral mRNA. The mutations are located within two distinct stem-loop structures in the 5' noncoding region of poliovirus RNA that constitute a major portion of a putative internal ribosome entry site. On the basis of our data derived from genetic and biochemical assays, the stability of one of the stem-loop structures appears to be essential for translation initiation via internal binding of ribosomes. However, the second stem-loop structure may function in a manner that requires base pairing and proper spacing between specific nucleotide sequences. By employing RNA electrophoretic mobility shift assays, an RNA-protein interaction was detected for this latter stem-loop structure that does not occur in RNAs containing mutations which perturb the predicted hairpin structure. Analysis of in vivo-selected virus revertants, in combination with mobility shift assays, suggests that extensive genetic rearrangement can lead to restoration of 5' noncoding region functions, possibly by the repositioning of specific RNA sequence or structure motifs.
Multiple intraluminal electrical impedancometry for recording of upper gastrointestinal motility: current results and further implications

This review focuses on current aspects of the novel technology of multiple intraluminal electrical impedance measurement. It presents methodological features, summarizes current results, and discusses potential implications for further research. The impedance technique assesses a bolus transport and its associated peristalsis. Validation studies showed a good analogy between physically deduced impedance characteristics and characteristics derived from cineradiography and manometry. From the impedance tracings, it is possible to distinguish between resting states, bolus transit, and wall contraction. Characteristics of a peristaltic wave can be obtained. In human studies, esophageal and small intestinal peristaltic patterns can quantitatively and qualitatively be assessed. A high resolution recording of bolus movements with interesting details of transport and mixing can be obtained. On the basis of several prior characterized impedance tracings duodenal contractile patterns have been classified, and the interdigestive and postprandial states characterized. For reflux evaluation the impedance technique was especially useful for the detection of nonacid gastroesophageal reflux, which is not detectable by pH monitoring. In summary, the main impact of the impedance technique is its capability to characterize esophageal and intestinal chyme transport. Important data on luminal chyme transport have been obtained. This technique is developing into an interesting investigative tool to complement standard techniques for study of upper GI motility, in particular for basic research.
Application of flow cytometry in pediatric hematology‐oncology

Applications of flow cytometry in pediatric cancers have expanded substantially in recent years. In acute leukemias, the commonest childhood cancer, flow cytometry can now define complex antigenic profiles that are associated with specific cytogenetic/molecular defects and can also directly identify BCR‐ABL fusion protein. Flow cytometry based scoring system has been described for diagnosis of myelodysplastic syndromes. In solid tumors, flow cytometry was previously used mainly to determine DNA content for prognosis; however, recent studies in children with neuroblastoma and Ewing sarcoma have identified its diagnostic utility. In this review, we will discuss the current and future applications of flow cytometry in pediatric hematology‐oncology. Pediatr Blood Cancer 2011;57:18–29. © 2011 Wiley‐Liss, Inc.