bluuebunny/crossref_metadata_2025_split huggingface dataset:
Surgical management of thoracolumbar kyphosis in mucopolysaccharidosis type 1 in a reference center
Authors: Abelin Genevois Kariman , Garin Christophe , Solla Federico , Guffon Nathalie , Kohler Rémi 
Published: June 2013

AbstractPurposeMucopolysaccharidosis (MPS) I is a rare autosomal recessive lysosomal storage disease. Thoracolumbar kyphosis is an early characteristic feature of the disease. Ossification failure in the anterosuperior quadrant of the vertebral body results in anterior dislocation. This study describes the surgical management of thoracolumbar kyphosis in MPS IH (Hurler syndrome) in a national reference center.MethodsAmong 72 MPS I patients followed in our institution, we treated surgically 14 MPS IH patients with severe thoracolumbar kyphosis. The decision was made after documented deformity progression. Mean age at surgery was 8 (3.5–15) years. Sagittal imbalance of the trunk was constant. One patient underwent extended fusion for associated scoliosis. We retrospectively reviewed 13 patients who underwent selective circumferential fusion at the thoracolumbar level.ResultsAverage preoperative kyphosis was +57.5°(+30°; +90°). Surgical correction of the kyphosis was about 66 % and maintained at final follow‐up. Fusion was obtained in all patients. Two patients required revision surgery consecutively to a previous posterior‐only fusion, as a significant loss of correction occurred. One patient presented delayed neurologic deficit secondarily to cardiac embolism. One patient died postoperatively from cardiorespiratory failure.ConclusionSurgery is necessary when kyphosis is progressive despite orthopedic management, aggravating the multifactorial trunk imbalance. Regarding our experience, circumferential arthrodesis should be recommended to achieve stable correction. Surgical management requires a multidisciplinary approach due to multisystemic failure and neurological risks specific to metabolic disorders.
Environmental factors controlling seed germination and seedling recruitment of Stipa bungeana on the Loess Plateau of northwestern China
Authors: Hu Xiao Wen , Zhou Zhi Qin , Li Ting Shan , Wu Yan Pei , Wang Yan Rong 
Published: June 2013

AbstractGermination studies are important for collecting information on field seedling recruitment, plant conservation and restoration. This study investigated the role of light, temperature, nitrogen, water stress and burial depth in controlling germination of Stipa bungeana seeds. S. bungeana seeds are photo‐inhibited; light significantly decreased seed germination regardless of temperature and water conditions. Seeds germinated at 10–30° C, and the highest germination was 72 % and 88 % at 20° C in light and dark, respectively. Thermal model analysis showed that presence of light significantly increased average thermal requirement [θT(50)] from 105°Cd to 186°Cd at sub‐optimal temperature, implying that light delays seed germination. Hydrotime model analysis showed that presence of light caused a shift in the median base water potential [Ψb(50)] from −0.68 to −0.26 MPa, which partly explains why light decreased both percentage and speed of germination, even at optimal conditions. As burial depth increased, seedling emergence initially increased and then decreased; the highest seedling emergence recruitment was 43 %, for seeds buried at a depth of 1 cm. Field observations showed that seedling emergence occurred primarily from July to September, and scarcely occurred from April to June. These results suggest that the light inhibitory effect is an adaptive mechanism that prevents S. bungeana seeds from germinating on the soil surface. To attain highest seedling establishment, seeds of S. bungeana should be sown at a soil depth of 1 cm prior to the rainy season, using seeds stored for 1 year.
Determination of Aroma Profiles of Olive Oils from Turkish Olive Cultivars
Authors: Bayrak Ali , Kıralan Mustafa , Kara Hasan Hüseyin 
Published: June 2013

AbstractThis study analyzed volatile aromatic compounds present in the oils of Turkish olive cultivars. The cultivars Gemlik, Ayvalık, Memecik, Domat, Uslu, Halhalı, Kilis yağlık, Nizip yağlık, Haşebi and Karamani were harvested from important Turkish olive‐growing locations, such as Balıkesir, Bursa, Manisa, Aydın, Muğla, İzmir, Kilis, Mersin, Hatay and Gaziantep during the 2007–2008 and 2008–2009 growing seasons. Olive samples were collected in two harvest years, at almost the same maturity stage, and processed under the same conditions. Headspace solid‐phase microextraction (HS‐SPME) was used to analyze volatile aroma compounds in the olive oil samples. Forty‐seven compounds were characterized and quantified using gas chromatography‐mass spectrometry/flame ionization detector (GC–MS/FID). The most abundant identified compounds were trans‐2‐hexenal, hexanal and 3‐methyl‐1‐butanol. The trans‐2‐hexenal, hexanal and 3‐methyl‐1‐butanol contents of oil samples varied between 0.86–67.15, 3.93–61.82 and 0.48–84.74 %, respectively.
Flexed-knee gait in children with cerebral palsy: A 10-year follow-up study
Authors: Haumont Thierry , Church Chris , Hager Shaun , Cornes Maria Julia , Poljak Dijana , Lennon Nancy , Henley John , Taylor Daveda , Niiler Tim , Miller Freeman 
Published: June 2013

Background While several studies have evaluated the short-term effectiveness of conservative and surgical treatment of flexed-knee gait in children with cerebral palsy (CP), few have explored the long-term outcomes using gait analysis. The purpose of this study was to examine, through gait analysis, the 10-year outcomes of flexed-knee gait in children with CP. Methods Ninety-seven children with spastic CP who walked with a flexed-knee gait underwent two gait evaluations [age 6.1 ± 2.1 and 16.2 ± 2.3 years, Gross Motor Function Classification System (GMFCS) I (12), II (45), III (37), IV (3)]. Limbs with knee flexion at initial contact >15° were considered walking with a flexed-knee gait and were included in the study ( n = 185). Kinematic data were collected using an eight-camera motion analysis system (Motion Analysis, Santa Rosa, CA). Surgical and therapeutic interventions were not controlled. Results A comparison between the two gait studies showed an overall improvement in gait at 10 years follow-up. Significant improvements were seen in knee flexion at initial contact, Gait Deviation Index (GDI), Gross Motor Function Measure (GMFM), and gait speed ( P < 0.01 for all). Outcome was also evaluated based on the severity of flexed-knee gait at the initial visit, with functional skills and overall gait (GDI) improving in all groups ( P < 0.01 for all). The group with a severe flexed-knee gait exhibited the most improvement, while subjects with a mild flexed-knee improved the least. Conclusions Children at a specialty hospital whose orthopedic care included gait analysis and multi-level surgery showed improvement of flexed-knee gait and gross motor function over a 10-year course, regardless of the initial severity.
A Preliminary Assessment of a Novel Pneumatic Unloading Knee Brace on the Gait Mechanics of Patients With Knee Osteoarthritis
Authors: Della Croce Ugo , Crapanzano Fausto , Li Ling , Kasi Patrick K. , Patritti Benjamin L. , Mancinelli Chiara , Hunter David J. , Stamenović Dimitrije , Harvey William F. , Bonato Paolo 
Published: June 2013

ObjectivesTo determine whether a knee brace incorporating inflatable air bladders can alter the net peak external knee adduction moment in persons with medial compartment knee osteoarthritis.DesignProspective cohort study.SettingMotion analysis laboratory.ParticipantsSubjects (n = 18) diagnosed with knee osteoarthritis as defined by the Diagnostic and Therapeutic Criteria Committee of the American Rheumatism Association.MethodsInstrumented gait analysis was performed while subjects walked with and without the knee brace. When subjects wore the knee brace, the air bladders were either uninflated or inflated to 7 psi. The net external knee adduction moment was obtained by subtracting the abduction moment produced by the knee brace (estimated using a finite element analysis model) from the external knee adduction moment (estimated using a camera‐based motion analysis system).Main Outcome MeasurementsThe net external knee adduction moment was compared across all testing conditions.ResultsA 7.6% decrease in net peak external knee adduction moment was observed when subjects wore the knee brace uninflated compared with when they did not wear the brace. Inflation of the bladders to 7 psi led to a 26.0% decrease in net peak external knee adduction moment.ConclusionsThe results of the study suggest that the effects of an unloading knee brace may be enhanced by incorporating inflatable air bladders into the design of the brace, thus leading to an improved correction of the excessive peak external knee adduction moment observed in patients with medial compartment knee osteoarthritis.
Symmetry-Defying Iron Pyrite (FeS2) Nanocrystals through Oriented Attachment
Authors: Gong Maogang , Kirkeminde Alec , Ren Shenqiang 
Published: June 2013

AbstractIron pyrite (fool's gold, FeS2) is a promising earth abundant and environmentally benign semiconductor material that shows promise as a strong and broad absorber for photovoltaics and high energy density cathode material for batteries. However, controlling FeS2 nanocrystal formation (composition, size, shape, stoichiometry, etc.) and defect mitigation still remains a challenge. These problems represent significant limitations in the ability to control electrical, optical and electrochemical properties to exploit pyrite's full potential for sustainable energy applications. Here, we report a symmetry-defying oriented attachment FeS2 nanocrystal growth by examining the nanostructure evolution and recrystallization to uncover how the shape, size and defects of FeS2 nanocrystals changes during growth. It is demonstrated that a well-controlled reaction temperature and annealing time results in polycrystal-to-monocrystal formation and defect annihilation, which correlates with the performance of photoresponse devices. This knowledge opens up a new tactic to address pyrite's known defect problems.
Non-Engineered Nanoparticles of C60
Authors: Deguchi Shigeru , Mukai Sada-atsu , Sakaguchi Hide , Nonomura Yoshimune 
Published: June 2013

AbstractWe discovered that rubbing bulk solids of C60between fingertips generates nanoparticles including the ones smaller than 20 nm. Considering the difficulties usually associated with nanoparticle production by pulverisation, formation of nanoparticles by such a mundane method is unprecedented and noteworthy. We also found that nanoparticles of C60could be generated from bulk solids incidentally without deliberate engineering of any sort. Our findings imply that there exist highly unusual human exposure routes to nanoparticles of C60and elucidating formation mechanisms of nanoparticles is crucial in assessing their environmental impacts.
Candy Wrapper for the Earth's Inner Core
Authors: Mattesini M. , Belonoshko A. B. , Tkalčić H. , Buforn E. , Udías A. , Ahuja R. 
Published: June 2013

AbstractRecent global expansion of seismic data motivated a number of seismological studies of the Earth's inner core that proposed the existence of increasingly complex structure and anisotropy. In the meantime, new hypotheses of dynamic mechanisms have been put forward to interpret seismological results. Here, the nature of hemispherical dichotomy and anisotropy is re-investigated by bridging the observations of PKP(bc-df) differential travel-times with the ironbcc/hcpelastic properties computed fromfirst-principlesmethods.TheCandy Wrappervelocity model introduced here accounts for a dynamic picture of the inner core (i.e., the eastward drift of material), where different iron crystal shapes can be stabilized at the two hemispheres. We show that seismological data are best explained by a rather complicated, mosaic-like, structure of the inner core, where well-separated patches of different iron crystals compose the anisotropic western hemispherical region and a conglomerate of almost indistinguishable iron phases builds-up the weakly anisotropic eastern side.
CRISPR interference: a structural perspective
Authors: Reeks Judith , Naismith James H. , White Malcolm F. 
Published: June 2013

CRISPR (cluster of regularly interspaced palindromic repeats) is a prokaryotic adaptive defence system, providing immunity against mobile genetic elements such as viruses. Genomically encoded crRNA (CRISPR RNA) is used by Cas (CRISPR-associated) proteins to target and subsequently degrade nucleic acids of invading entities in a sequence-dependent manner. The process is known as ‘interference’. In the present review we cover recent progress on the structural biology of the CRISPR/Cas system, focusing on the Cas proteins and complexes that catalyse crRNA biogenesis and interference. Structural studies have helped in the elucidation of key mechanisms, including the recognition and cleavage of crRNA by the Cas6 and Cas5 proteins, where remarkable diversity at the level of both substrate recognition and catalysis has become apparent. The RNA-binding RAMP (repeat-associated mysterious protein) domain is present in the Cas5, Cas6, Cas7 and Cmr3 protein families and RAMP-like domains are found in Cas2 and Cas10. Structural analysis has also revealed an evolutionary link between the small subunits of the type I and type III-B interference complexes. Future studies of the interference complexes and their constituent components will transform our understanding of the system.
Brown adipose tissue: development, metabolism and beyond
Authors: Schulz Tim J. , Tseng Yu-Hua 
Published: June 2013

Obesity represents a major risk factor for the development of several of our most common medical conditions, including Type 2 diabetes, dyslipidaemia, non-alcoholic fatty liver, cardiovascular disease and even some cancers. Although increased fat mass is the main feature of obesity, not all fat depots are created equal. Adipocytes found in white adipose tissue contain a single large lipid droplet and play well-known roles in energy storage. By contrast, brown adipose tissue is specialized for thermogenic energy expenditure. Owing to its significant capacity to dissipate energy and regulate triacylglycerol (triglyceride) and glucose metabolism, and its demonstrated presence in adult humans, brown fat could be a potential target for the treatment of obesity and metabolic syndrome. Undoubtedly, fundamental knowledge about the formation of brown fat and regulation of its activity is imperatively needed to make such therapeutics possible. In the present review, we integrate the recent advancements on the regulation of brown fat formation and activity by developmental and hormonal signals in relation to its metabolic function.
From Proto-Hungarian SOV to Old Hungarian Top Foc V X
Authors: Kiss Katalin É. 
Published: June 2013

This paper argues that Hungarian underwent a word order change from SOV to Top Foc V X* prior to its documented history beginning in 1192. Proto-Hungarian SOV is reconstructed primarily on the basis of shared constructions of archaic Old Hungarian, and Khanty and Mansi, the sister languages of Hungarian. The most likely scenario of the change from head-final to head-initial was the spreading of right dislocation, and the reanalysis of right dislocated elements by new generations of speakers as arguments in situ. In Hungarian — as opposed to Khanty and Mansi — right dislocation was facilitated by the extension of differential object marking to all direct objects. The change in basic word order initiated the restructuring of other parts of Hungarian grammar as well, which is a still ongoing process.
[As of June 2015, this article is available as Open Access under a CC-BY-NC 4.0 license.]
Genome-wide identification and predictive modeling of tissue-specific alternative polyadenylation
Authors: Hafez Dina , Ni Ting , Mukherjee Sayan , Zhu Jun , Ohler Uwe 
Published: June 2013

Abstract
Motivation: Pre-mRNA cleavage and polyadenylation are essential steps for 3′-end maturation and subsequent stability and degradation of mRNAs. This process is highly controlled by cis-regulatory elements surrounding the cleavage/polyadenylation sites (polyA sites), which are frequently constrained by sequence content and position. More than 50% of human transcripts have multiple functional polyA sites, and the specific use of alternative polyA sites (APA) results in isoforms with variable 3′-untranslated regions, thus potentially affecting gene regulation. Elucidating the regulatory mechanisms underlying differential polyA preferences in multiple cell types has been hindered both by the lack of suitable data on the precise location of cleavage sites, as well as of appropriate tests for determining APAs with significant differences across multiple libraries.
Results: We applied a tailored paired-end RNA-seq protocol to specifically probe the position of polyA sites in three human adult tissue types. We specified a linear-effects regression model to identify tissue-specific biases indicating regulated APA; the significance of differences between tissue types was assessed by an appropriately designed permutation test. This combination allowed to identify highly specific subsets of APA events in the individual tissue types. Predictive models successfully classified constitutive polyA sites from a biologically relevant background (auROC = 99.6%), as well as tissue-specific regulated sets from each other. We found that the main cis-regulatory elements described for polyadenylation are a strong, and highly informative, hallmark for constitutive sites only. Tissue-specific regulated sites were found to contain other regulatory motifs, with the canonical polyadenylation signal being nearly absent at brain-specific polyA sites. Together, our results contribute to the understanding of the diversity of post-transcriptional gene regulation.
Availability: Raw data are deposited on SRA, accession numbers: brain SRX208132, kidney SRX208087 and liver SRX208134. Processed datasets as well as model code are published on our website: http://www.genome.duke.edu/labs/ohler/research/UTR/
Contact: uwe.ohler@duke.edu
Effects of ocean acidification on hatch size and larval growth of walleye pollock (Theragra chalcogramma)
Authors: Hurst Thomas P. , Fernandez Elena R. , Mathis Jeremy T. 
Published: June 2013

Abstract
Hurst, T. P., Fernandez, E. R., and Mathis, J. T. 2013. Effects of ocean acidification on hatch size and larval growth of walleye pollock (Theragra chalcogramma). – ICES Journal of Marine Science, 70: 812–822. Rising atmospheric concentrations of CO2 are predicted to decrease the pH of high-latitude oceans by 0.3–0.5 units by 2100. Because of their limited capacity for ion exchange, embryos and larvae of marine fishes are predicted to be more sensitive to elevated CO2 than juveniles and adults. Eggs and larvae of walleye pollock (Theragra chalcogramma) were incubated across a broad range of CO2 levels (280–2100 µatm) to evaluate sensitivity in this critical resource species. Slightly elevated CO2 levels (∼450 µatm) resulted in earlier hatching times, but differences among egg batches were greater than those observed across CO2 treatments. Egg batches differed significantly in size-at-hatch metrics, but we observed no consistent effect of CO2 level. In three independent experiments, walleye pollock were reared at ambient and elevated CO2 levels through the early larval stage (to ∼30 days post-hatch). Across trials, there were only minor effects of CO2 level on size and growth rate, but fish in the ambient treatments tended to be slightly smaller than fish reared at elevated CO2 levels. These results suggest that growth potential of early life stages of walleye pollock is resilient with respect to the direct physiological effects of ocean acidification.
Evaluating deepwater fisheries management strategies using a mixed-fisheries and spatially explicit modelling framework
Authors: Marchal Paul , Vermard Youen 
Published: June 2013

Abstract
Marchal, P., and Vermard, Y. 2013. Evaluating deepwater fisheries management strategies using a mixed-fisheries and spatially explicit modelling framework. – ICES Journal of Marine Science, 70: 768–781. We have used in this study a spatially explicit bioeconomic modelling framework to evaluate management strategies, building in both data-rich and data-limited harvest control rules (HCRs), for a mix of deepwater fleets and species, on which information is variable. The main focus was on blue ling (Molva dypterygia). For that species, both data-rich and data-limited HCRs were tested, while catch per unit effort (CPUE) was used either to tune stock assessments, or to directly trigger management action. There were only limited differences between the performances of both HCRs when blue ling biomass was initialized at the current level, but blue ling recovered more quickly with the data-rich HCR when its initial biomass was severely depleted. Both types of HCR lead, on average, to a long-term recovery of both blue ling and saithe (Pollachius virens) stocks, and some increase in overall profit. However, that improvement is not sufficient to guarantee sustainable exploitation with a high probability. Blue ling CPUE did not always adequately reflect trends in biomass, which mainly resulted from fleet dynamics, possibly in combination with density-dependence. The stock dynamics of roundnose grenadier (Coryphaenoides rupestris), black scabbardfish (Aphanopus carbo) and deepwater sharks (Centrophorus squamosus and Centroscymnus coelolepis) were little affected by the type of HCR chosen to manage blue ling.
Krill biomass and aggregation structure in relation to tidal cycle in a penguin foraging region off the Western Antarctic Peninsula
Authors: Bernard Kim S. , Steinberg Deborah K. 
Published: June 2013

Abstract
Bernard, K. S., and Steinberg, D. K. 2013. Krill biomass and aggregation structure in relation to tidal cycle in a penguin foraging region off the Western Antarctic Peninsula. – ICES Journal of Marine Science, 70: 834–849
Antarctic krill are a key component of the diet of Adélie penguins inhabiting the Western Antarctic Peninsula (WAP), yet our understanding of the variability of krill distribution patterns within nearshore penguin feeding grounds is limited. A recent study of the foraging patterns of penguins breeding in the northern WAP suggests that tidal phase plays a role in foraging distance. We used acoustics to examine biomass and aggregation structure of krill in the penguin foraging grounds off Palmer Station during diurnal and semi-diurnal tides. Nearshore, integrated krill biomass during diurnal tides was significantly higher than during semi-diurnal tides. Krill aggregations were also shallower, closer together, and larger in dimension during diurnal tides. Conversely, krill aggregations had higher volumetric biomass and abundance during semi-diurnal tides. Further offshore, at the head of the Palmer Deep canyon, krill aggregations were similar to those observed nearshore during diurnal tides (i.e. shallow, close together, and large in dimension). Since krill aggregation structure strongly influences availability as a potential prey source, we suggest that foraging behavior of Adélie penguins in this region is strongly linked to the variability in nearshore krill aggregation structure as well as biomass.