bluuebunny/crossref_metadata_2025_split huggingface dataset:
A novel SACS mutation results in non‐ataxic spastic paraplegia and peripheral neuropathy
Authors: Gregianin E. , Vazza G. , Scaramel E. , Boaretto F. , Vettori A. , Leonardi E. , Tosatto S. C. E. , Manara R. , Pegoraro E. , Mostacciuolo M. L. 
Published: June 2013

Background and purposeMutations in the SACS gene are commonly associated with autosomal recessive spastic ataxia of Charlevoix‐Saguenay (ARSACS), a complex neurodegenerative disorder characterized by progressive degeneration of the cerebellum and spinal cord tracts. The aim of this study was to identify the genetic cause of the disease in an Italian family with spastic paraplegia and peripheral neuropathy.MethodsAffected subjects were subjected to a comprehensive neurological examination including electromyography and brain magnetic resonance imaging. Genetic studies included exclusion of known disease genes, genome‐wide linkage analysis using high density single nucleotide polymorphism genotyping and candidate gene sequencing.ResultsMolecular analyses revealed a novel missense mutation in the SACS gene (c.11,104A>G) occurring in a homozygous state in patients and absent in 700 Italian control chromosomes. The mutation led to the amino acid substitution p.Thr3702Ala in the sacsin protein, in a possible protein−protein interaction site of UBE3A binding domain.ConclusionThis study broadens the genetic spectrum of SACS mutations and expands the clinical ARSACS phenotype suggesting that the SACS gene can be considered in patients with non‐canonical ARSACS clinical presentations.
The Confusions and Exuberances of Biopolitics
Authors: Rutherford Paul , Rutherford Stephanie 
Published: June 2013

AbstractThis paper is the first of a two‐part exploration of the significance of biopolitics to the study of geography. Our first paper surveys the emergent field of biopolitics by looking at the philosophical underpinnings of the concept and some of the ways in which scholars, including geographers, have utilized the concept as an analytical tool. Throughout the papers, we make the argument for a greater degree of empirical rigor and conceptual clarity in the way the term biopolitics is deployed and operationalized.
Molecular cloning, sequencing and structural studies of granulocyte–macrophage colony‐stimulating factor (GM‐CSF) from Indian water buffalo (Bubalus bubalis)
Authors: Sugumar Thennarasu , Pugalenthi Ganesan , Harishankar Murugesan , Dhinakar Raj G 
Published: June 2013

SummaryGranulocyte–macrophage colony‐stimulating factor (GM‐CSF) is a cytokine that is essential for growth and development of progenitors of granulocytes and monocytes/macrophages. In this study, we report molecular cloning, sequencing and characterization of GM‐CSF from Indian water buffalo, Bubalus bubalis. In addition, we performed sequence and structural analysis for buffalo GM‐CSF. Buffalo GM‐CSF has been compared with 17 mammalian GM‐CSFs using multiple sequence alignment and phylogenetic tree. Three‐dimensional model for buffalo GM‐CSF and human receptor complex was built using homology modelling to study cross‐reactivity between two species. Detailed analysis was performed to study GM‐CSF interface and various interactions at the interface.
Has Europe Been Catching Up? An Industry Level Analysis of Venture Capital Success over 1985–2009
Authors: Kräussl Roman , Krause Stefan 
Published: June 2013

AbstractAfter nearly two decades of US leadership during the 1980s and 1990s, are Europe's venture capital (VC) markets in the 2000s finally catching up regarding the provision of financing and successful exits, or is the performance gap as wide as ever? Are we amid an overall VC performance slump with no encouraging news? We attempt to answer these questions by tracking over 40,000 VC‐backed firms stemming from six industries in 13 European countries and the USA between 1985 and 2009; determining the type of exit – if any – each particular firm's investors choose for the venture.
Laser Vermilion Ablation for Actinic Cheilitis
Authors: DAVID LAURENCE M. 
Published: June 2013

A new surgical procedure employing a carbon dioxide laser for treating actinic cheilitis is described. Eight patients having biopsy‐proven actinic cheilitis and one patient having clinically evident actinic cheilitis but biopsy‐proven superficial basal cell carcinoma were treated by vermilion ablation using the carbon dioxide laser.After an average follow‐up period of 34 months (27 to 38 months), no patient has had a recurrence of cheilitis. Normal contour of the treated lip has been preserved in all patients, while none has had significant post‐treatment scarring.
Keloids: Scar Revision
Authors: BROWN LOUIS A. , PIERCE HAROLD E. 
Published: June 2013

Abstract
The treatment of keloids can be a long‐term, and at times, vexing, dermatologic surgical management problem arising in both white and non‐white patients. This paper reviews a number of conventional, as well as novel, therapeutic approaches for the management of this disease of uncontrolled scar growth. Additionally, fundamental aspects of this disease are covered, including clinical, histologic, biochemical, immunologic, endocrinologic, and epidemiologic factors.An understanding of these latter aspects may be useful to the clinician in realizing the best possible therapeutic results. It must be emphasized that, regardless of the technique employed, an observation period of at least 2 years is necessary to effectively limit the chance and degree of recurrence.
Residency and Racial/Ethnic Differences in Weight Status and Lifestyle Behaviors Among US Youth
Authors: Kenney Mary Kay , Wang Jing , Iannotti Ron 
Published: June 2013

AbstractPurposeElevated risk for obesity is found in rural environments and in some minority populations. It is unclear whether living in rural or nonmetropolitan areas and being a minority compound the risk of obesity beyond that of either factor acting alone. Our purpose was to examine adolescent obesity in light of the potential concomitant influences of race/ethnicity, residency, and obesity‐related lifestyle behaviors.MethodsWe assessed obesity prevalence, physical activity, consumption of fatty snack foods, and screen time in 8,363 US adolescents based on variation in race/ethnicity and residency. Descriptive, bivariate, and multivariate statistics were used to: (1) calculate race‐ and residency‐based rates of obesity and obesity‐related lifestyle behaviors and (2) generate race‐ and residency‐based obesity odds ratios as a function of those same behaviors.FindingsThe results indicated that nonmetropolitan black youth had the highest risk of obesity (26%), rate of consuming fatty snack foods on more than 2 days/week (86%), and rate of spending more than 2 hours/day in screen time (91%) compared to white metropolitan youth. Compared to their metropolitan counterparts, black nonmetropolitan youth had greater odds of being obese if they exercised less than daily (1.71 times), ate fatty snack foods on more than 2 days/week (1.65 times), or spent more than 2 hours/day in screen time (1.64 times).ConclusionsRace/ethnicity and residency may have a compounding effect on the risk of obesity. Prevention and intervention must be viewed in a socioecological framework that recognizes the importance of culture and community on obesity‐related behaviors.
Light noble gases in 12 meteorites from the Omani desert, Australia, Mauritania, Canada, and Sweden
Authors: Leya I. , Ammon K. , Cosarinsky M. , Dalcher N. , Gnos E. , Hofmann B. , Huber L. 
Published: June 2013

AbstractWe measured the concentrations and isotopic compositions of He, Ne, and Ar in 14 fragments from 12 different meteorites: three carbonaceous chondrites, six L chondrites (three most likely paired), one H chondrite, one R chondrite, and one ungrouped chondrite. The data obtained for the CV3 chondrites Ramlat as Sahmah (RaS) 221 and RaS 251 support the hypothesis of exposure age peaks for CV chondrites at approximately 9 Ma and 27 Ma. The exposure age for Shişr 033 (CR chondrite) of 7.3 Ma is also indicative of a possible CR chondrite exposure age peak. The three L chondrites Jiddat al Harasis (JaH) 091, JaH 230, and JaH 296, which are most likely paired, fall together with Hallingeberg into the L chondrite exposure age peak of approximately 15 Ma. The two L chondrites Shelburne and Lake Torrens fall into the peaks at approximately 40 Ma and 5 Ma, respectively. The ages for Bassikounou (H chondrite) and RaS 201 (R chondrite) are approximately 3.5 Ma and 5.8 Ma, respectively. Six of the studied meteorites show clear evidence for 3He diffusive losses, the deficits range from approximately 17% for one Lake Torrens aliquot to approximately 45% for RaS 211. The three carbonaceous chondrites RaS 221, RaS 251, and Shişr 033 all have excess 4He, either of planetary or solar origin. However, very high 4He/20Ne ratios occur at relatively low 20Ne/22Ne ratios, which is unexpected and needs further study. The measured 40Ar ages fit well into established systematics. They are between 2.5 and 4.5 Ga for the carbonaceous chondrites, older than 3.6 Ga for the L and H chondrites, and about 2.4 Ga for the R chondrite as well as for the ungrouped chondrite. Interestingly, none of our studied L chondrites has been degassed in the 470 Ma break‐up event. Using the amount of trapped 36Ar as a proxy for noble gas contamination due to terrestrial weathering we are able to demonstrate that the samples studied here are not or only very slightly affected by terrestrial weathering (at least in terms of their noble gas budget).
Maternal milk DHA content predicts cognitive performance in a sample of 28 nations
Authors: Lassek William Day , Gaulin Steven J.C. 
Published: June 2013

AbstractConvergent evidence from neuronal biology and hominin brain hypertrophy suggests that omega‐3 fatty acids are a limiting resource for neural and cognitive development in Homo sapiens, and therefore that children from populations with higher omega‐3 availability should display superior cognitive performance. Using multiple regression, we tested this prediction in a sample of 28 countries, with Programme for International Student Assessment (PISA) math scores in 2009 as an index of cognitive performance, and country‐specific breast milk levels of omega‐3 docosahexaenoic acid (DHA) as an index of omega‐3 availability. Breast milk DHA makes a highly significant contribution to math scores (β = 0.462, P = 0.006), greater in magnitude than the control variables of per capita Gross Domestic Product (PCGDP) and educational expenditures per pupil. Together, dietary fish (positively) and total fat (negatively) explain 61% of the variance in maternal milk DHA in a larger sample of 39 countries.
A prospective study of maternal fatty acids, micronutrients and homocysteine and their association with birth outcome
Authors: Wadhwani Nisha S. , Pisal Hemlata R. , Mehendale Savita S. , Joshi Sadhana R. 
Published: June 2013

AbstractOur earlier studies both in animals and in humans have indicated that micronutrients (folic acid, vitamin B12) and long‐chain polyunsaturated fatty acids, especially docosahexaenoic acid (DHA), are interlinked in the one‐carbon cycle, which plays an important role in fetal ‘programming’ of adult diseases. The present study examines the levels of maternal and cord plasma fatty acids, maternal folate, vitamin B12 and homocysteine in healthy mothers at various time points during pregnancy and also examine an association between them. A longitudinal study of 106 normal pregnant women was carried out, and maternal blood was collected at three time points, viz., T1 = 16–20th week, T2 = 26–30th week and T3 = at delivery. Cord blood was collected at delivery. Fatty acids were estimated using a gas chromatograph. Levels of folate, vitamin B12 and homocysteine were estimated by the chemiluminescent microparticle immunoassay (CMIA) technology. Maternal plasma folate (P < 0.05), vitamin B12 (P < 0.01) and DHA (P < 0.05) levels were lowest, while maternal homocysteine levels were highest (P < 0.01) at T3. There was a negative association between maternal DHA and homocysteine at T2 (P < 0.05) and T3 (P < 0.01). There was a positive association between plasma DHA in maternal blood at T3 and cord blood. Furthermore, there was a positive association between maternal folate and vitamin B12 at T3 and baby weight, whereas maternal homocysteine at T1 were inversely associated with baby weight at delivery. Our study provides evidence for the associations of folic acid, vitamin B12, homocysteine with DHA and baby weight, suggesting that a balanced dietary supplementation of folate–vitamin B12–DHA during pregnancy may be beneficial.
Effect of complementary feeding with lipid‐based nutrient supplements and corn–soy blend on the incidence of stunting and linear growth among 6‐ to 18‐month‐old infants and children in ruralMalawi
Authors: Mangani Charles , Maleta Kenneth , Phuka John , Cheung Yin Bun , Thakwalakwa Chrissie , Dewey Kathryn , Manary Mark , Puumalainen Taneli , Ashorn Per 
Published: June 2013

AbstractLow nutritional value of complementary foods is associated with high incidence of childhood growth stunting in low‐income countries. This study was done to test a hypothesis that dietary complementation with lipid‐based nutrient supplements (LNS) promotes linear growth and reduces the incidence of severe stunting among at‐risk infants. A total of 840 6‐month‐old healthy infants in ruralMalawi were enrolled to a randomised assessor‐blinded trial. The participants received 12‐month supplementation with nothing, milk–LNS, soy–LNS, or corn–soy blend (CSB). Supplements provided micronutrients and approximately 280 kcal energy per day. Outcomes were incidence of severe and very severe stunting [length‐for‐agez‐score, (LAZ) < −3.00 and <−3.50, respectively], and change inLAZ. The incidence of severe stunting was 11.8%, 8.2%, 9.1% and 15.5% (P = 0.098) and that of very severe stunting 7.4%, 2.9%, 8.0% and 6.4% (P = 0.138) in control, milk–LNS, soy–LNSandCSBgroups, respectively. Between 9 and 12 months of age, the mean change inLAZwas −0.15, −0.02, −0.12 and −0.18 (P = 0.045) for control, milk–LNS, soy–LNSandCSBgroups, respectively. There was no significant between‐group difference in linear growth during other age‐intervals. Although participants who received milk–LNShad the lowest incidence of severe and very severe stunting, the differences between the groups were smaller than expected. Thus, the results do not provide conclusive evidence on a causal association between theLNSsupplementation and the lower incidence of stunting. Exploratory analyses suggest that provision of milk–LNS, but not soy–LNSpromotes linear growth among at‐risk infants mainly between 9 and 12 months of age.
The Training of Jobseekers by Non‐profit Organizations: An Analysis Based on Data from the Upper Normandy Region of France
Authors: Bailly Franck , Chapelle Karine 
Published: June 2013

AbstractThere is now an extensive academic literature on non‐profit organizations (NPOs). The hypothesis that these organizations are different from for‐profit organizations (FPOs) is one that is frequently adopted. Nevertheless, a number of recent studies have suggested that many factors may cause NPOs to lose their specificity. The aim of this paper is to assess the influence of these variables empirically with regard to the continuing training sector. Our results lead us to conclude that, while some of these variables do indeed have an impact on the behavior of NPOs, there is sufficient evidence to conclude that they do behave differently from FPOs.
Hardcore Re‐visioned: Reading and Misreading in Sonic Youth, 1987–8
Authors: Heetderks David 
Published: June 2013

AbstractThis article uses Harold Bloom's concept of revision, defined as a reading that is simultaneously a re‐interpretation, to examine how the alternative band Sonic Youth transformed the hardcore style. Sonic Youth embraced hardcore in the early 1980s as a means of expanding beyond their no‐wave origins, but because of a cultural gulf between them and other hardcore bands, their successful appropriation of hardcore necessarily involved fragmentation and ironic reversal of its stylistic elements, combined with formally unorthodox means of re‐integrating those elements.Examining lyrics, scalar subsets, gestural motions on the guitar, timbre, references to formal archetypes and cues for pitch centricity, this article shows revision in two Sonic Youth songs from the late 1980s. ‘Silver Rocket’ (1988) fragments riffs characteristic of hardcore and substitutes a large‐scale constructive principle for musical narrative in its verse and chorus. ‘White Kross’ (1987) transforms hardcore's characteristic stance against authority, creating instead a sense of internal conflict both lyrically and musically. I present the analyses in order to provide a foundation for examining other post‐punk songs and explaining Sonic Youth's cultural significance in the alternative‐rock scene.
Indole‐3‐butyric acid induces lateral root formation via peroxisome‐derived indole‐3‐acetic acid and nitric oxide
Authors: Schlicht Markus , Ludwig‐Müller Jutta , Burbach Christian , Volkmann Dieter , Baluska Frantisek 
Published: June 2013

Summary

Controlled plant growth requires regulation through a variety of signaling molecules, including steroids, peptides, radicals of oxygen and nitrogen, as well as the ‘classical’ phytohormone groups. Auxin is critical for the control of plant growth and also orchestrates many developmental processes, such as the formation of new roots. It modulates root architecture both slowly, through actions at the transcriptional level and, more rapidly, by mechanisms targeting primarily plasma membrane sensory systems and intracellular signaling pathways. The latter reactions use several second messengers, including Ca2+, nitric oxide (NO) and reactive oxygen species (ROS).

Here, we investigated the different roles of two auxins, the major auxin indole‐3‐acetic acid (IAA) and another endogenous auxin indole‐3‐butyric acid (IBA), in the lateral root formation process of Arabidopsis and maize. This was mainly analyzed by different types of fluorescence microscopy and inhibitors of NO production.

This study revealed that peroxisomal IBA to IAA conversion is followed by peroxisomal NO, which is important for IBA‐induced lateral root formation.

We conclude that peroxisomal NO emerges as a new player in auxin‐induced root organogenesis. In particular, the spatially and temporally coordinated release of NO and IAA from peroxisomes is behind the strong promotion of lateral root formation via IBA.
Fiscal Foresight, Limited Information and the Effects of Government Spending Shocks
Authors: Fragetta Matteo , Gasteiger Emanuel 
Published: June 2013

AbstractWe quantify the impact of government spending shocks in the US. Thereby, we control for fiscal foresight, a specific limited information problem (LIP) by utilizing the narrative approach. Moreover, we surmount the generic LIP inherent in vector autoregressions (VARs) by a factor‐augmented VAR (FAVAR) approach. We find that a positive deficit‐financed defence shock raises output by more than in a VAR (e.g. 2.61 vs. 2.04 for peak multipliers). Furthermore, our evidence suggests that consumption is crowded in. These results are robust to variants of controlling for fiscal foresight and reveal the crucial role of the LIP in fiscal VARs.
Changes in villus‐like projections of corneocytes from the facial skin in normal infants with or without infantile eczema; useful parameter to assess barrier function
Authors: Naoko Ota , Satoshi Hirakawa , Fukuyoshi Mori , Mitsuyoshi Honda 
Published: June 2013

Background/PurposeSkin barrier function changes rapidly during the first year of life in infants. Measuring skin barrier function in infants is difficult because of the burden on patients and their parents or guardians. A simple method for assessing barrier function in infants that can be performed quickly and would enable swift treatment and skin care management.MethodsWe used laser microscopy to investigate villus‐like projections (VPs) observed on the lower surface of corneocytes obtained on tape strips from 18 normal Japanese infants at the ages of 1, 3, and 6 months. We also performed transepidermal water loss and corneocyte size measurements. A dermatologist clinically assessed the cutaneous findings. We rapidly measured skin barrier function changes during the first 6 months of life in infants.ResultsDuring the study period, very slight erythema and pityriasis were the primary reasons for skin rash, which were generally most severe at 3 months. The results showed that barrier function declined at approximately 3 months of age in nearly all subjects. Therefore, we found that normal barrier function of infantile skin becomes temporarily impaired after birth.ConclusionWe conclude that even in patients with very mild skin rashes, multiple objective indices of skin barrier function deteriorate simultaneously. Observation of VPs on the surface of corneocytes using laser microscopy is a rapid, painless, and effective method of monitoring skin barrier function in infants, and VP score offers a useful index for assessing barrier function.
An objective assessment of melanin in vitiligo skin treated with Balneo PUVA therapy
Authors: Hegyi V. , Petrovajová M. , Novotný M. 
Published: June 2013

BackgroundVisual clinical methods of skin color evaluation for diagnostic purposes are so far mostly subjective and thus inaccurate. We present a modified method of melanin amount measurement based on diffuse reflectance spectroscopy (DRS). This method is non‐invasive and objective, and allows easy quantification and comparison of melanin levels.Material and methodsSkin pigmentation was measured by DRS method in 0‐18 year old patients at the Department of Pediatric Dermatovenerology, School of Medicine Comenius University Bratislava. Patients were treated for their vitiligo by Balneo PUVA treatment twice weekly. Each patient had measured his remittance spectra from the treated vitiliginous skin before the treatment was started, after 10 irradiations of Balneo PUVA and at the end of the treatment after 25 irradiations of Balneo PUVA. In our study as a reference skin for spectroscopic assessment of melanin in vivo was used the averaged remittance spectra (measured on the inner arm) from the sample of 10 albino patients. The remittance spectra obtained from the vitiligo patients were ratioed against the newly described remittance reference albino skin. We exploited the linear behavior of the spectral curve in the 620‐720 nm interval (significant for melanin absorption) and used the slope of the regression line to compute the quantification index α.ResultsBy clinical examination before the Balneo PUVA therapy, after the 10th dose of Balneo PUVA therapy as well as at the end of the complete course of Balneo PUVA therapy (after 25 irradiations) we recorded a marked increase of pigmentation in all treated patients for their vitiligo. In each patient the values of melanin quantification angle α were calculated. Statistically we found a significant difference between the melanin quantification angle α in vitiliginous skin before, during the 10th dose of treatment and after the treatment. Similar significant difference was also observed between treated and non‐involved skin. We could confirm a clear association between clinical visual examination of treated vitiligo lesions, objective data collected by DRS and melanin quantification angle α.ConclusionsBy using a new standard for the reference skin (albino skin) we could more exactly compare melanin levels in different subjects. Our proposed melanin quantification angle α expresses the extent of the difference in melanin levels between the examined skin lesions. We successfully used this index to quantify the variations of melanin (progress of repigmentation) throughout different stages of treatment of the same lesion and also to objectively evaluate the final effect of the therapy. In the present study, we showed that the diffuse reflectance spectroscopy (DRS) may be suitable method to measure skin colour and the content of human skin melanin in vivo.