bluuebunny/crossref_metadata_2025_split huggingface dataset:
Studies on glycoprotein metabolism in different types of sphingolipidosis
Authors: de Gasperi R. , Rapelli S. , Lindi C. , Berra B. 
Published: December 2005

AbstractAbnormalities in glycoprotein composition have been demonstrated in brain tissue from patients with GM1 gangliosidosis and Niemann‐Pick disease and in spleen from patients with Gaucher's disease. The mechanism producing these glycoprotein abnormalities is probably different in each disease.
Chemical compositions of acid mucopolysaccharides in urine and tissues of patients with multiple sulphatase deficiency
Authors: Eto Y. , Tokoro T. , Ito F. , Herschkowitz N. N. 
Published: December 2005

AbstractDetailed chemical analyses of the acid mucopolysaccharides of liver, brain and urine in multiple sulphatase deficiency have shown heparin sulphate to be the major component.
Glucose metabolism in a child with 3‐hydroxy‐3‐methylglutaryl‐coenzyme a lyase deficiency
Authors: François B. , Bachmann C. , Schutgens R. B. H. 
Published: December 2005

AbstractA child with 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency suffered from fasting hypoglycaemia and impaired ketone body formation.
Inheritance of transcobalamin II (TC II) in two families with TC II deficiency and related immunodeficiency
Authors: Fràter‐Schröder M. , Hitzig W. H. , Sacher M. 
Published: December 2005

AbstractTranscobalamin II (TC II) is an essential transport protein for vitamin B12 in blood. TC II can be separated into isoproteins by polyacrylamide gel electrophoresis. This method was used in combination with a specific radioimmunosorbent technique to evaluate genetic variants and inheritance of TC II‐deficient genes in relatives of two children with congenital TC II deficiency. Both patients presented with impairment of haematopoietic and immunological functions. Seven heterozygous individuals for TC II deficiency, who are clinically normal, were detected in the two families. Two out of seven could be identified unambiguously by TC II isoprotein analysis, as carriers of a deficient gene, which does not express functional TC II. Application of this new method to detect heterozygous carriers of the deficient gene provides a valuable addition to genetic counselling.
Biochemical findings in a patient with lipoprotein lipase deficiency (Hyperlipoproteinaemia Type I)
Authors: Abeling N. G. G. M. , Bakker H. D. , van Gennip A. H. , Jansen H. , Vázquez de la Cruz A. 
Published: December 2005

AbstractAn early diagnosed case of hyperlipoproteinaemia type I is presented. Besides the major criteria, which were met, unexpected changes in the serum lipoprotein pattern on heparin injection are reported, suggesting a possible role of liver lipase in HDL metabolism.
Urinary pyrimidine excretion in arginase deficiency
Authors: Naylor E. W. , Cederbaum S. D. 
Published: December 2005

AbstractA high‐performance liquid‐chromatographic method was used to separate and identify uracil, uridine, pseudouridine and orotic acid after preliminary extraction in two patients (McKusick 20780). Urinary uracil excretion was 10–35 times normal in both patients with arginase deficiency. Uridine and orotic acid, not normally detected, were excreted in large amounts and were directly influenced by protein intake. Their excretions were correlated with urinary arginine excretion. Urinary uracil levels remained consistently high and showed minimal variations with increased protein intake or urinary arginine levels.The measurement of urinary pyrimidines appears to be useful for the detection, differential diagnosis and dietary monitoring of patients with urea cycle disorders. The data presented extends this observation to include patients with arginase deficiency.
Renal histidinuria
Authors: Kamoun P. P. , Parvy P. , Cathelineau L. , Meyer B. 
Published: December 2005

AbstractOne patient with abnormal histidinuria and a normal plasma histidine concentration is presented. This patient had myoclonic seizures like another patient previously reported with histidinuria. The effect of a peroral histidine loading test was studied. The relationship between renal histidinuria and neurological abnormalities is discussed.
Combined deficiency of β‐galactosidase and neuraminidase: Three affected siblings in a French family
Authors: Maire I. , Nivelon‐Chevallier A. 
Published: December 2005

AbstractThree affected siblings from France have been described with a combined deficiency of β‐galactosidase and neuraminidase. Oligosaccharides were found in urine and the enzyme activities determined in leukocytes and cultured fibroblasts. Further characterization of the defect in this family is needed.
Climatic and intertrophic effects detected in 10‐year population dynamics of biological control of the arrowhead scale by two parasitoids in southwestern Japan
Authors: Tuda Midori , Matsumoto Takashi , Itioka Takao , Ishida Norio , Takanashi Masaaki , Ashihara Wataru , Kohyama Mitsuko , Takagi Masami 
Published: December 2005

AbstractRelative effects of weather and three‐trophic interactions were studied for a classical biological control system consisting of the arrowhead scale Unaspis yanonensis, known formerly as a serious pest of the Satsuma mandarin orange Citrus unshiu, and its two introduced parasitoids, Coccobius fulvus and Aphytis yanonensis. Yearly population responses of the three insect species on a per‐tree basis for up to 10 years at two orange groves were analyzed by general linear models, with a backward stepwise procedure, to select among abiotic (summer/winter temperature and rainfall) and biotic (densities of the three insect species and orange bearing in the previous years) independent variables. Temperature positively affected the arrowhead scale and the two parasitoids. A negative correlation of rainfall was detected for all three insect species. Mandarin fruiting showed negative delayed density dependence, thereby supporting the observed alternate bearing phenomenon in mandarins, presumably due to physiological imbalance triggered by climatic factors. The arrowhead scale was negatively correlated with fruit production in the preceding years, possibly due to reduced resistance in subsequent years of mast fruiting. We found a negative correlation of the arrowhead scale with Coccobius only in a single grove and none with Aphytis. Thus, it appears that bottom‐up forces may be more important than top‐down control by the parasitoids in the post‐transient phase of this system.
The influence of seasonal precipitation and temperature regimes on lake levels in the northeastern United States during the Holocene
Authors: Shuman Bryan , Donnelly Jeffrey P. 
Published: December 2005

AbstractAMS–dated sediment cores combined with ground–penetrating radar profiles from two lakes in southeastern Massachusetts demonstrate that regional water levels rose and fell multiple times during the Holocene when the known climatic controls (i.e., ice extent and insolation) underwent unidirectional changes. The lakes were lowest between 10,000 and 9000 and between 5500 and 3000 cal yr B.P. Using a heuristic moisture-budget model, we explore the hypothesis that changes in seasonal precipitation regimes, driven by monotonic trends in ice extent and insolation, plausibly explain the multiple lake-level changes. Simulated lake levels resulting from low summer precipitation rates match observed low lake levels of 10,000–9000 cal yr B.P., whereas a model experiment that simply shifts the seasonality of the modern Massachusetts precipitation regime (i.e., moving the peak monthly precipitation from winter to summer) produces levels that are ∼2 m lower than today as observed for 5500–3000 cal yr B.P. The influence of the Laurentide ice sheet could explain dry summers before ca. 8000 cal yr B.P. A later shift from a summer-wet to a winter-wet moisture-balance regime could have resulted from insolation-driven changes in the influence of the Bermuda subtropical high. Temperature changes probably further modified lake levels by affecting snowmelt and transpiration.
Broadcasts of Deliberative Polls: Aspirations and Effects
Authors: FISHKIN JAMES S. , LUSKIN ROBERT C. 
Published: December 2005

Every face-to-face Deliberative Poll (DP) to date has been the subject of a television broadcast. We consider these broadcasts a helpful adjunct to the design – a way of motivating both the random sample and the policy experts and policy makers to attend, of educating the broader public about the issues, and, perhaps, of nudging public opinion in the direction of the results. In ‘Rickety Bridges’, John Parkinson examines just one of these broadcasts, Channel 4's on the DP on the future of Britain's National Health Service (NHS) in 1998. Applying his coding of the contents to other DP broadcasts might or might not yield similar results, but we are happy to assume, for argument's sake, that it would. If DP broadcasts are generally doing what he describes the NHS DP broadcast as doing, they are doing pretty well, at least as far as the distribution of coverage is concerned. It is Parkinson's notion of what they should be doing that is mistaken. As a result, his critique is fundamentally misguided.THE AIMS OF DP BROADCASTSParkinson's critique rests on an inappropriate standard. His central claim is that Channel 4's broadcast of the NHS DP did not replicate the participants' experience. Of course it did not. No broadcast could ever give viewers the same experience they would have if they were actually part of the DP's on-site, weekend-long deliberations. The broadcast in that case would have to be weekend-long, and there would actually have to be multiple broadcasts – as many as there are participants – since every participant's experience is different.
Of Scale and Straw Men: A Reply to Fishkin and Luskin
Authors: PARKINSON JOHN 
Published: December 2005

I am grateful to James Fishkin and Robert Luskin for taking the time to consider and respond to my remarks on the dangers of relying on the news media to form a bridge between participants and non-participants in deliberative events. Clearly we are in agreement that there is a difference between deliberations as experienced by participants and a television audience. However, Fishkin and Luskin have misrepresented my aims and claims about that difference, and thus their response is less telling than it might have been, directed as it is at a man of straw.At the outset, let me emphasize that the purpose ofmyresearch note was not to subject deliberative polls to a thorough critique: one case study would indeed have been an inadequate foundation for such work. Rather, the case study was a device used to highlight a feature common to many attempts to put deliberative democratic principles into practice, not just the deliberative poll (DP). The underlying issue is, to state it again, one of scale: millions of people cannot strictly deliberate together. Walzer proposes a maximum number of twenty individuals, although the literature on small groups suggests that the actual limit may be between five and seven, both which seem disturbingly small for purportedly democratic processes. Even if the deliberative limit were several hundred, however, the scale issue still arises and causes problems for the democratic legitimacy of such events: how can their decisions be binding on others when those others have not had the opportunity to have their views transformed by the encounter with the better argument?
Organic cucumber production in the greenhouse: A case study from Turkey
Authors: Tuzel Y. , Gul A. , Tuncay O. , Anac D. , Madanlar N. , Yoldas Z. , Gumus M. , Tuzel I.H. , Engindeniz S. 
Published: December 2005

AbstractOrganic farming seems to be a solution in environmentally sensitive zones such as the protection zone of Tahtalı Dam, which supplies fresh water to Izmir, the third largest city in Turkey. Greenhouse vegetable production is the major agricultural activity in this area (Menderes Township). However, due to the pollution risk from agriculture, governmental authorities have issued a regulation discouraging the use of chemicals within the collection basin of the dam. An on-farm project was conducted in 2001 to introduce organic greenhouse vegetable production in the Tahtalı Dam preservation area and to serve as a model for other areas in Turkey facing similar ecological problems. Two irrigation levels and four organic fertilizers were tested on the growth of cucumbers (Cucumis sativus cv. Sardes). The four fertilizers were two rates of farmyard and poultry manure (30 and 50 tons ha−1) with and without two organic fertilizers based on either bacteria or algae. During the growing period, pests and diseases were monitored weekly and preparations allowed in organic agriculture were used when necessary. Plant diseases did not create any significant problem, but the pest population, in particular spidermites, increased. No significant differences in cucumber yield were found between the fertilizer and irrigation treatments tested. However, it should be noted that yields from the addition of 30 tons ha−1 of farmyard or poultry manure and organic fertilizers were the same as those for 50 tons ha−1 of manure alone. The average total yield was 13 kg m−2, within an acceptable range for short-term crop production in this region, but 55% lower than a conventional substrate culture trial nearby. However, due to reduced production costs and a price premium for organic products, the organic produce yielded a net return of US$1.12–1.79 per m2 whereas that for conventional bag culture produce was US$0.55 per m2. Our results indicate that, under present conditions, organic greenhouse cucumber production has less environmental impact and is more economic than conventional bag culture, due to reduced chemical input and reduced operating and initial investment costs.
Farmer research: Conventional experiences and guidelines for alternative agriculture and multi-functional agro-ecosystems
Authors: Wortmann C.S. , Christiansen A.P. , Glewen K.L. , Hejny T.A. , Mulliken J. , Peterson J.M. , Varner D.L. , Wortmann S. , Zoubek G.L. 
Published: December 2005

AbstractCrop producers are challenged to operate profitably, use resources efficiently, meet high standards of quality and protect the environment, while sustaining rural economies and societies. Cropping systems are generally fine-tuned and improved through changes that have small effects which can often be verified only through research. The processes and successes of two farmer research projects were studied. Results of these studies, information from other sources and the authors' reflections on their own experiences were integrated to develop guidelines for the implementation of farmer research projects for alternative agriculture and multi-functional agro-ecosystems with diverse stakeholders. Surveys were mailed to 118 farmers currently or previously participating in a farmer research project, and to 15 advisors. Responses show that involvement in a farmer research project was profitable, stimulating, enjoyable and worthwhile, despite a substantial time requirement. Tillage and soil fertility research had greater impact on annual farm profit than research on other topics. Farmers and advisors emphasized the importance of the farmers' roles in identification of research topics, research planning and implementation, and interpretation of the results. Replicated trials conducted over 2 or 3 years were recognized as necessary to adequately verify practices for the corn–soybean rotation of eastern Nebraska, USA. Such trials may need to be complemented with alternative research approaches for improving alternative agriculture and multi-functional agro-ecosystems where knowledge about some system components is relatively scarce and there is a need to evaluate long-term effects. In conclusion, organized farmer research is an efficient means to cropping system improvement. Guidelines are given for initiation and implementation of farmer research projects.
The adoption and impact of management intensive rotational grazing (MIRG) on Connecticut dairy farms
Authors: Foltz Jeremy , Lang Gillis 
Published: December 2005

AbstractManagement intensive rotational grazing (MIRG) has garnered a great deal of interest in recent years as a method for returning profitability to Northeastern dairy farms. This work uses a random sample of Connecticut dairy farmers to estimate a binary choice adoption model and then cost, productivity, and profit functions that control for the adoption choice. MIRG adopters are shown to be more educated and have less rented agricultural land (a proxy for lack of access to land within a short distance of the barn). MIRG adoption had no significant effects on costs and productivity, nor did it lower profits, per cow. Evidence was found, however, to suggest that full adopters of the technology had more profitable farms than partial adopters. These results also show the importance of controlling for the different characteristics of adopters when evaluating the returns to animal grazing.
Is the Arabian Gulf really such a lowspot of biodiversity?: Scaling effects and management implications
Authors: Price A. R. G. , Izsak C. 
Published: December 2005

Analysis of echinoderm datasets for the Arabian Gulf and Red Sea region reveals lower species richness in the Gulf at 4 different spatial scales: Point, Sample, Large area and Biogeographical province. Species richness correlates positively with spatial scale in both regions, as does endemism, although levels of the latter are comparable for both regions.
Values of taxonomic distinctness (Δ*), a relatedness measure based on average properties, are very similar for the Gulf and the Red Sea over all spatial scales. This might seem surprising, given that (i) the Gulf's environment is highly stressful, and (ii) the Red Sea is one the world's top 10 coral reef high diversity areas, and the habitat associated with most echinoderm records for this region, but not for the Gulf. These findings may partly reflect the behaviour of Δ* to ‘saturate’ (reach maximal values as sampling/spatial scale increases) much sooner than species richness, endemism and other ‘species’ measures.
Our results suggest the Gulf may not be the lowspot of biodiversity generally acclaimed, especially if a broad set of measures is utilised. Biodiversity is an important constituent of ecosystem health and criterion for determining where conservation attention should be targeted. Until the functional/ecological significance of Δ* is better understood, management implications of our study's findings remain unclear. Further attention should also be given to β-diversity, the change in species composition along a gradient. We also stress that any conclusions drawn from the study are tentative, pending compilation and analysis of datasets for other groups.
The evolution of hyperactivity, impulsivity and cognitive diversity
Authors: Williams Jonathan , Taylor Eric 
Published: December 2005

The evolutionary status of attention deficit/hyperactivity disorder (ADHD) is central to assessments of whether modern society has created it, either physically or socially; and is potentially useful in understanding its neurobiological basis and treatment. The high prevalence of ADHD (5–10%) and its association with the seven-repeat allele of DRD4, which is positively selected in evolution, raise the possibility that ADHD increases the reproductive fitness of the individual, and/or the group. However, previous suggestions of evolutionary roles for ADHD have not accounted for its confinement to a substantial minority. Because one of the key features of ADHD is its diversity, and many benefits of population diversity are well recognized (as in immunity), we study the impact of groups' behavioural diversity on their fitness. Diversity occurs along many dimensions, and for simplicity we choose unpredictability (or variability), excess of which is a well-established characteristic of ADHD.Simulations of the Changing Food group task show that unpredictable behaviour by aminorityoptimizes results for the group. Characteristics of suchgroup exploration tasksare risk-taking, in which costs are borne mainly by the individual; and information-sharing, in which benefits accrue to the entire group. Hence, this work is closely linked to previous studies of evolved altruism.We conclude that even individually impairing combinations of genes, such as ADHD, can carry specific benefits for society, which can be selected for at that level, rather than being merely genetic coincidences with effects confined to the individual. The social benefits conferred by diversity occur both inside and outside the ‘normal’ range, and these may be distinct. This view has the additional merit of offering explanations for the prevalence, sex and age distribution, severity distribution and heterogeneity of ADHD.
Highly engineered mesoporous structures for optical processing
Authors: Parker G.J , Charlton M.D.B , Zoorob M.E , Baumberg J.J , Netti M.C , Lee T 
Published: December 2005

Arranging periodic, or quasi-periodic, regions of differing refractive index in one, two, or three dimensions can form a unique class of mesoporous structures. These structures are generally known as photonic crystals, or photonic quasicrystals, and they are the optical analogue of semiconducting materials. Whereas a semiconductor's band structure arises from the interaction of electron or hole waves with an arrangement of ion cores, the photonic crystal band structure results from the interaction of light waves with an arrangement of regions of differing refractive index.
What makes photonic crystals highly attractive to the optical engineer is that we can actually place the regions of differing refractive index in a pattern specifically tailored to produce a given optical function, such as an extremely high dispersion, for example. That is, we can define the geometrical arrangement of the dielectric foam to provide us with the form of band structure we require for our optical functionality.
In this paper, the optical properties and applications of these highly engineered mesoporous dielectrics will be discussed.