bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deletion of a single allele of thePex11βgene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain
Authors: Ahlemeyer Barbara , Gottwald Magdalena , Baumgart-Vogt Eveline 
Published: September 2011

SUMMARYImpaired neuronal migration and cell death are commonly observed in patients with peroxisomal biogenesis disorders (PBDs), and in mouse models of this diseases. In Pex11β-deficient mice, we observed that the deletion of a single allele of the Pex11β gene (Pex11β+/− heterozygous mice) caused cell death in primary neuronal cultures prepared from the neocortex and cerebellum, although to a lesser extent as compared with the homozygous-null animals (Pex11β−/− mice). In corresponding brain sections, cell death was rare, but differences between the genotypes were similar to those found in vitro. Because PEX11β has been implicated in peroxisomal proliferation, we searched for alterations in peroxisomal abundance in the brain of heterozygous and homozygous Pex11β-null mice compared with wild-type animals. Deletion of one allele of the Pex11β gene slightly increased the abundance of peroxisomes, whereas the deletion of both alleles caused a 30% reduction in peroxisome number. The size of the peroxisomal compartment did not correlate with neuronal death. Similar to cell death, neuronal development was delayed in Pex11β+/− mice, and to a further extent in Pex11β−/− mice, as measured by a reduced mRNA and protein level of synaptophysin and a reduced protein level of the mature isoform of MAP2. Moreover, a gradual increase in oxidative stress was found in brain sections and primary neuronal cultures from wild-type to heterozygous to homozygous Pex11β-deficient mice. SOD2 was upregulated in neurons from Pex11β+/− mice, but not from Pex11β−/− animals, whereas the level of catalase remained unchanged in neurons from Pex11β+/− mice and was reduced in those from Pex11β−/− mice, suggesting a partial compensation of oxidative stress in the heterozygotes, but a failure thereof in the homozygous Pex11β−/− brain. In conclusion, we report the alterations in the brain caused by the deletion of a single allele of the Pex11β gene. Our data might lead to the reconsideration of the clinical treatment of PBDs and the common way of using knockout mouse models for studying autosomal recessive diseases.
Differential segmental strain during active lengthening in a large biarticular thigh muscle during running
Authors: Carr Jennifer A. , Ellerby David J. , Marsh Richard L. 
Published: September 2011

SUMMARYThe iliotibialis lateralis pars postacetabularis (ILPO) is the largest muscle in the hindlimb of the guinea fowl and is thought to play an important role during the stance phase of running, both absorbing and producing work. Using sonomicrometry and electromyography, we examined whether the ILPO experiences differential strain between proximal, central and distal portions of the posterior fascicles. When the ILPO is being lengthened while active, the distal portion was found to lengthen significantly more than either the proximal or central portions of the muscle. Our data support the hypothesis that the distal segment lengthened farther and faster because it began activity at shorter sarcomere lengths on the ascending limb of the length–tension curve. Probably because of the self-stabilizing effects of operating on the ascending limb of the length–tension curve, all segments reached the end of lengthening and started shortening at the same sarcomere length. During shortening, this similarity in sarcomere length among the segments was maintained, as predicted from force–velocity effects, and shortening strain was similar in all segments. The differential active strain during active lengthening is thus ultimately determined by differences in strain during the passive portion of the cycle. The sarcomere lengths of all segments of the fascicles were similar at the end of active shortening, but after the passive portion of the cycle the distal segment was shorter. Differential strain in the segments during the passive portion of the cycle may be caused by differential joint excursions at the knee and hip acting on the ends of the muscle and being transmitted differentially by the passive visco-elastic properties of the muscle. Alternatively, the differential passive strain could be due to the action of active or passive muscles in the thigh that transmit force to the IPLO in shear. Based on basic sarcomere dynamics we predict that differential strain is more likely to occur in muscles undergoing active lengthening at the beginning of contraction than those undergoing only shortening.
Role of the lateral line mechanosensory system in directionality of goldfish auditory evoked escape response
Authors: Mirjany Mana , Preuss Thomas , Faber Donald S. 
Published: September 2011

SUMMARYGoldfish (Carassius auratus) escape responses to sudden auditory stimuli are mediated by a pair of reticulospinal neurons, the Mauthner (M-) cells, which integrate mechanosensory inputs from the inner ear and the lateral line (LL) to initiate a fast directional response away from the aversive stimulus. This behavior is context dependent; when near an obstruction the fish may rather turn towards the sound to avoid hitting the object. Mechanisms underlying this directionality remain unknown. Here we investigate the contribution of the LL system to auditory evoked escapes and provide behavioral evidence that it transmits stimulus – and environmental-dependent information that determines the initial response direction of the escape. We quantified escape latency, probability and directionality following abrupt sound stimuli before and after removal of the entire LL with 0.03 mmol l–1 cobalt chloride (CoCl2), 0.002% gentamicin or selective posterior LL nerve (pLLn) transection. CoCl2 significantly increased escape onset latency without affecting probability and reduced open field directionality from 77% to chance, 52%. This effect on directionality was also observed with gentamicin. Transection of the pLLn had no effect on directionality, indicating the anterior LL nerve (aLLn) afferents are more likely to transmit directional information to the M-cell. When the fish were near a wall, the error rate was quadrupled by both CoCl2 and pLLn transection. Visual elimination had no influence on directionality unless combined with LL elimination.
Carotenoid intake does not affect immune-stimulated oxidative burst in greenfinches
Authors: Sild Elin , Sepp Tuul , Männiste Marju , Hõrak Peeter 
Published: September 2011

SUMMARY
Carotenoid-based integument colouration is extremely widespread in the animal kingdom. It has been hypothesized that carotenoid colouration is used for communicating the health status of the bearers because carotenoids are efficient immunomodulators or antioxidants. However, the latter argument has been recently debated and the mechanisms by which carotenoids modulate immunity or oxidative balance are poorly known. We performed an experiment on wild-caught captive greenfinches, passerine birds with carotenoid-based plumage colouration, in order to test whether dietary carotenoid supplementation affects immune-stimulated oxidative burst of phagocytes in the whole blood and humoral immune response to a novel antigen, Brucella abortus (BA). Additionally, we tested whether immune stimulation with bacterial lipopolysaccharide (LPS) affects blood carotenoid levels. We thus tested the effects of carotenoids on the oxidative burst of phagocytes under neutral conditions and during in vivo immune challenge. LPS injection depleted plasma carotenoids, indicating involvement of these phytochemicals in the immune response. However, we did not find any evidence that manipulation of carotenoid intake had modulated anti-BA antibody production, LPS-stimulated oxidative burst of phagocytes, or basal levels of circulating reactive oxygen species. This indicates that carotenoid intake does not affect endogenous production of reactive oxygen species by immune cells. This finding is consistent with the view that carotenoids are unlikely to provide a direct link between oxidative stress and colouration. However, it remains to be tested whether the oxidative burst of phagocytes induced in our experiment actually inflicts oxidative damage and whether carotenoids play a role in the attenuation of such potential damages.
Synthesis and Characterization of Ni-doped Silica Membranes Prepared Using a Hybrid Sol–Gel/CVD Method
Authors: Araki Sadao , Yano Hiroyuki , Tanaka Shunsuke , Miyake Yoshikazu 
Published: September 2011

Abstract
Improving the hydrogen permeation flux, while maintaining high hydrogen selectivity, is important for reducing membrane areas and enhancing membrane reactor (MR) performance. We have attempted to enhance the hydrogen permeation through the addition of nickel, which will improve both hydrogen adsorption and pore size control. The silica layer was deposited with high selectivity, using an extended counter-diffusion CVD method.
Homeostatic Interactions at the Front of Migration Control the Integrity and the Efficiency of a Migratory Glial Chain
Authors: Berzsenyi Sara , Kumar Arun , Giangrande Angela 
Published: September 2011

In metazoans, cell migration often occurs in a collective manner: the cells move while physically and functionally connected to their neighbors. The coordinated and timely movement of the cells eventually ensures the proper organization of tissues, and deregulation in such a process contributes to the development of severe diseases. Thus, understanding the cellular mechanisms underlying coordinated cell movement is of great interest in basic and medical science.The developingDrosophilawing provides an excellent model to follow the chain migration of glial cellsin vivo. Cells at the tip of the glial collective have been shown to control the timely movement of the chain. In the present study, we show that while pioneers trigger chain migration, they cannot move as single cells. We also show that isolating cell clusters at the chain tip restores the formation of smaller migratory communities. Interestingly, the migratory efficiency of thesede novoformed communities depends on the number of cells and progressively improves as the size of the cluster increases. Thus, homeostatic events at the migratory front control community integrity, efficiency, and coordination, emphasizing the importance of interactions and cell counting in fine-tuning collective processes.
Neonatal presentation of familial glucocorticoid deficiency resulting from a novel splice mutation in the melanocortin 2 receptor accessory protein
Authors: Jain V , Metherell L A , David A , Sharma R , Sharma P K , Clark A J L , Chan L F 
Published: September 2011

BackgroundFamilial glucocorticoid deficiency (FGD) is a rare autosomal recessive disorder characterised by isolated glucocorticoid deficiency. Mutations in the ACTH receptor/melanocortin 2 receptor (MC2R), the MC2R accessory protein (MRAP) or the STAR protein (STAR) cause FGD types 1, 2 and 3, respectively, accounting for ∼50% of all cases.Patient and methodsWe report a neonate of Indian origin, who was diagnosed with FGD in the first few days of life. He presented with hypoglycaemic seizures and was noted to have generalised intense hyperpigmentation and normal male genitalia. Biochemical investigations revealed hypocortisolaemia (cortisol 0.223 μg/dl; NR 1–23 μg/dl) and elevated plasma ACTH (170 pg/ml). Serum electrolytes, aldosterone and plasma renin activity were normal. Peak cortisol following a standard synacthen test was 0.018 μg/dl. He responded to hydrocortisone treatment and continues on replacement. Patient DNA was analysed by direct sequencing. The effect of the novel mutation was assessed by an in vitro splicing assay using wild type and mutant heterologous minigenes.ResultsA novel homozygous mutation c.106+2_3dupTA was found in the MRAP gene. Both parents were heterozygous for the mutation. In an in vitro splicing assay, the mutation resulted in the skipping of exon 3.ConclusionWe have identified a novel MRAP mutation where disruption of the intron 3 splice-site results in a prematurely terminated translation product. This protein (if produced) would lack the transmembrane domain that is essential for MC2R interaction. We predict that this would cause complete lack of ACTH response thus explaining the early presentation in this case.
The Conserved Foot Domain of RNA Pol II Associates with Proteins Involved in Transcriptional Initiation and/or Early Elongation
Authors: García-López M Carmen , Pelechano Vicent , Mirón-García M Carmen , Garrido-Godino Ana I , García Alicia , Calvo Olga , Werner Michel , Pérez-Ortín José E , Navarro Francisco 
Published: September 2011

Abstract
RNA polymerase (pol) II establishes many protein–protein interactions with transcriptional regulators to coordinate different steps of transcription. Although some of these interactions have been well described, little is known about the existence of RNA pol II regions involved in contact with transcriptional regulators. We hypothesize that conserved regions on the surface of RNA pol II contact transcriptional regulators. We identified such an RNA pol II conserved region that includes the majority of the “foot” domain and identified interactions of this region with Mvp1, a protein required for sorting proteins to the vacuole, and Spo14, a phospholipase D. Deletion of MVP1 and SPO14 affects the transcription of their target genes and increases phosphorylation of Ser5 in the carboxy-terminal domain (CTD). Genetic, phenotypic, and functional analyses point to a role for these proteins in transcriptional initiation and/or early elongation, consistent with their genetic interactions with CEG1, a guanylyltransferase subunit of the Saccharomyces cerevisiae capping enzyme.
Noninflammatory Pseudotumor Simulating Venous Thrombosis After Metal-on-Metal Hip Resurfacing
Authors: Maurer-Ertl Werner , Friesenbichler Joerg , Liegl-Atzwanger Bernadette , Kuerzl Gerda , Windhager Reinhard , Leithner Andreas 
Published: September 2011

Metal-on-metal hip resurfacing has become a widespread procedure, especially in young, physically active patients. Pseudotumor is a new complication that can occur after hip resurfacing and metal-on-metal total hip arthroplasty (THA).
This article presents a case of a 37-year-old woman who underwent metal-on-metal resurfacing of the left hip for symptomatic osteoarthritis. Twelve months following implantation, the patient reported painless swelling of the left lower leg. There was no clinical evidence of a deep venous thrombosis. Ultrasound and computed tomography showed a solid cystic lesion in the iliopsoas muscle, which communicated with the hip joint and compressed the external iliac vein. As a consequence, the cystic lesion was resected marginally. A few months later, the patient reported some discomfort in the groin and symptoms of instability, metallic clicking, and a restricted range of motion. Clinical and radiological examination revealed normal findings. Determining the serum concentration of cobalt and chromium revealed high increased levels of these metal ions. Ten months following excision of the pseudotumor, the patient reported recurrent swelling of the left lower leg. Computed tomography of the affected area showed a lobulated cystic formation; therefore, a relapse was suspected. At the second revision, the mass was excised and the implant was revised to a conventional ceramic-on-ceramic THA. At 30-month follow-up, the patient was doing well and there were no signs of local recurrence. Additionally, the metal ion levels of cobalt and chromium in the blood had significantly decreased.
Bowen Measure From Heteroclinic Points
Authors: Killough D. B. , Putnam I. F. 
Published: September 2011

Abstract We present a new construction of the entropy-maximizing, invariant probability measure on a Smale space (the Bowen measure). Our construction is based on points that are unstably equivalent to one given point, and stably equivalent to another, i.e., heteroclinic points. The spirit of the construction is similar to Bowen's construction from periodic points, though the techniques are very different. We also prove results about the growth rate of certain sets of heteroclinic points, and about the stable and unstable components of the Bowen measure. The approach we take is to prove results through direct computation for the case of a Shift of Finite type, and then use resolving factor maps to extend the results to more general Smale spaces.
Reaction Time‐based Detection of Concealed Information in Relation to Individual Differences in Executive Functioning
Authors: Visu‐Petra George , Miclea Mircea , Visu‐Petra Laura 
Published: September 2011

SummaryExecutive functioning has been shown to play an essential role in a person's ability to accurately and efficiently execute deceptive responses. The present study relates individual differences in executive functions and anxiety to the accuracy and latency of deceptive responses in a reaction time (RT)‐based Concealed Information Test with pictorial stimuli extracted from a mock crime scenario. Results indicated that the pictorial RT‐based Concealed Information Test successfully differentiated between guilty and innocent participants. In terms of executive functions, set‐shifting and inhibition were directly related to deception accuracy and speed, respectively. However, enhanced underlying working memory skills (both verbal and spatial) were associated with longer RTs for item classification. No strong associations were noted with anxiety dimensions. The implications of these findings are discussed in relation to neurocognitive models of deception and to the process of deception detection. Copyright © 2011 John Wiley & Sons, Ltd.
Dynamics in Using Different Question Types in Estonian Police Interviews of Children
Authors: Kask Kristjan 
Published: September 2011

SummaryThe present study examined Estonian police officers' interviewing tactics with children in cases of physical and/or sexual abuse, as there are no published studies conducted in a country formally part of the Soviet Union. Also, the dynamics in using different question types within interviews was examined. Investigative interviews with 66 children (mean age 8 years 10 months, range 4 to 14 years) were transcribed and analyzed. Option‐posing and direct questions were most frequently employed, but invitations produced significantly more information. Four to seven‐year‐old children were asked more suggestive questions than older children. As interviews progressed, the average number of explanations and general invitations per interview decreased, whereas the number of suggestive and option‐posing questions increased. This study suggests that like police officers in other countries, Estonian investigators require training in structured interviewing methods to increase the number of invitations and reduce reliance upon option‐posing and direct questions. Copyright © 2011 John Wiley & Sons, Ltd.
Do Non‐Verbal Aids Increase the Effectiveness of ‘Best Practice’ Verbal Interview Techniques? An Experimental Study
Authors: Salmon Karen , Pipe Margaret‐Ellen , Malloy Alana , Mackay Katherine 
Published: September 2011

SummaryTwo experiments examined the effectiveness of non‐verbal interview aids as means of increasing the amount of information children report about an event under conditions designed to mimic their use in the field. In the first study, 27 5–7‐year‐old children took part in an event, and 7–10 days later were interviewed using the National Institute of Child Health and Human Development Protocol interview followed by an opportunity to draw the event or complete puzzles and, in turn, a second verbal interview. New information was reported following both drawing and puzzles and accuracy declined in both conditions, but drawing did not differentially influence recall. In the second experiment, dolls or human figure diagrams were introduced to clarify children's (N = 53) reports of touch as recommended in by some professionals, with a verbal interview serving as a control. Props did not increase the amount of information reported compared with best practice verbal techniques, but nor did they elevate errors. The findings support the use of a second recall attempt, but do not support the use of non‐verbal aids, even when these are used following professional recommendations. Copyright © 2011 John Wiley & Sons, Ltd.
Pediatric regional examination of the musculoskeletal system: A practice‐ and consensus‐based approach
Authors: Foster Helen , Kay Lesley , May Carl , Rapley Tim 
Published: September 2011

AbstractObjectiveCompetent examination of the pediatric musculoskeletal (MSK) system is a vital component of clinical assessment of children with MSK presentations. The aim was to develop a regional MSK examination for school‐age children that is age appropriate and reflects clinical practice.MethodsQualitative and quantitative analyses involving video observation of clinical examination technique, systematic review, and expert consensus were employed to reveal descriptions, frequencies, and variations in technique for joint regions in various clinical scenarios. Systematic review and data from clinical observation were combined with feedback from a group of pediatric MSK experts through a web‐based survey. All results were collated and discussed by consensus development groups to derive the pediatric Regional Examination of the Musculoskeletal System (pREMS).ResultsA total of 48 pediatric MSK expert clinicians were involved to derive pREMS. Systematic review revealed a paucity of evidence about regional pediatric MSK examination. Video observations of MSK examinations (a total of 2,901 maneuvers) performed by pediatric MSK experts (n = 11 doctors and 8 therapists) of 89 school‐age children attending outpatient clinics in 7 UK pediatric rheumatology centers were followed by semistructured interviews with 14 of 19 clinicians. Video observation showed variation in examination techniques, most frequently at the hip and knee in the context of mechanical and inflammatory clinical scenarios.ConclusionpREMS is the first practice‐ and consensus‐based regional pediatric MSK examination for school‐age children. The structured approach is an important step toward improved pediatric MSK clinical skills relevant to clinical training.
Identification of phenotypes with different clinical outcomes in knee osteoarthritis: Data from the osteoarthritis initiative
Authors: Knoop Jesper , van der Leeden Marike , Thorstensson Carina A. , Roorda Leo D. , Lems Willem F. , Knol Dirk L. , Steultjens Martijn P. M. , Dekker Joost 
Published: September 2011

AbstractObjectiveTo identify subgroups or phenotypes of knee osteoarthritis (OA) patients based on similarities of clinically relevant patient characteristics, and to compare clinical outcomes of these phenotypes.MethodsData from 842 knee OA patients of the Osteoarthritis Initiative were used. A cluster analysis method was performed, in which clusters were formed based on similarities in 4 clinically relevant, easily available variables: severity of radiographic OA, lower extremity muscle strength, body mass index, and depression. Univariable and multivariable regression analyses were used to compare phenotypes on clinical outcomes (pain and activity limitations), taking into account possible confounders.ResultsFive phenotypes of knee OA patients were identified: “minimal joint disease phenotype,” “strong muscle phenotype,” “nonobese and weak muscle phenotype,” “obese and weak muscle phenotype,” and “depressive phenotype.” The “depressive phenotype” and “obese and weak muscle phenotype” showed higher pain levels and more severe activity limitations than the other 3 phenotypes.ConclusionFive phenotypes based on clinically relevant patient characteristics can be identified in the heterogeneous population of knee OA patients. These phenotypes showed different clinical outcomes. Interventions may need to be tailored to these clinical phenotypes.