bluuebunny/crossref_metadata_2025_split huggingface dataset:
Biotin‐responsive immunoregulatory dysfunction in multiple carboxylase deficiency
Authors: Munnich A. , Fischer A. , Saudubray J. M. , Griscelli C. , Coude F. X. , Ogier H. , Charpentier C. , Frezal J. 
Published: December 2005

AbstractMultiple carboxylase deficiency could be associated with defects of the immunoregulatory system. A boy with documented multiple carboxylase deficiency was found to display a fatty acid and biotin‐responsive impairment of his lymphocyte suppressive activityin vitro. Furthermore, prostaglandin E2 (PGE2), a major product of unsaturated fatty acid elongation required forin vitro activation of the immunoregulatory system, was found to be very low in the patient's monocytes. Finally, PGE2 monocytic production responded well to biotin therapy. PGE2 deficiency could result from the combination of an essential fatty acid deficiency with an impaired elongation of unsaturated fatty acids possibly due to a defect of the biotin‐dependent acetyl‐CoA carboxylase reaction.
Corrective factor of bloom syndrome: Further characteristics
Authors: Bartram C. R. , Rüdiger H. W. 
Published: December 2005

AbstractBloom syndrome is an autosomal recessive inherited disease characterizedin vitro by chromosomal instability and an increased rate of sister chromatid exchanges (SCE). Co‐cultivation of Bloom syndrome and normal fibroblasts results in correction of chromosomal instability in Bloom syndrome. We report our studies of the effects of co‐cultivation on homozygous Bloom cells with heterozygous cells. Additionally, we used fibroblasts from a patient with Fanconi anaemia and with xeroderma pigmentosum.
Glycopeptidosis: A new inherited disorder of glycoconjugate metabolism affecting the central nervous system
Authors: Federico A. , Guazzi G. , Fruschelli C. , Palladini G. , Medoloago‐Albani L. , Michalski J. C. , Strecker G. 
Published: December 2005

AbstractA patient with progressive neurological deterioration and myoclonus epilepsy showed an increased excretion of glycoprotein in the urine and storage material in lymphocytes and fibroblasts. This is probably a new disorder of glycoprotein metabolism.
Subcellular distribution of lysosomal hydrolases in mucolipidosis III and a mucolipidosis III variant
Authors: Kalaydjieva L. , Cantz M. 
Published: December 2005

AbstractThe subcellular distribution of lysosomal hydrolases and of [35S]sulphate‐labelled storage materials in cultured fibroblasts from typical and variant mucolipidosis III patients shows that these disorders probably have different pathogenetic mechanisms.
Abnormal cobalamin metabolism in a case of juvenile pernicious anaemia with neurological symptoms
Authors: Linnell J. C. , Quadros E. V. , England J. M. , Down M. C. , Reynolds E. H. 
Published: December 2005

AbstractStudies of cobalamin metabolism in case of juvenile pernicious anaemia during haematological remission showed that while plasma cobalamins were normal there was some depletion of erythrocyte cobalamins. In addition, the plasma distribution of apo‐ and holo‐transcobalamins was markedly abnormal, holo‐TCII being increased, yet the patient's plasma promoted less cellular synthesis of adenosylcobalamin by cultured lymphocytes than did plasma from a control subject. The results suggest that holo‐TCII may in this case have been ineffective and provide further evidence for the importance of TCII in intracellular synthesis of adenosylcobalamin.
Partial HGPRT‐deficiency in a patient with the Lesch‐Nyhan syndrome: Evidence for a structural mutation affecting the PRPP binding site
Authors: Rijksen G. , Staal G. E. J. , van der Vlist M. J. M. , Beemer F. A. , Troost J. , van Laarhoven J. P. R. M. , de Bruyn C. H. M. M. 
Published: December 2005

AbstractThe residual HGPRT activity in a patient with the Lesch‐Nyhan syndrome was 10–15% in haemolysate and about 30% in fibroblast lysate. Due to a structural mutation affecting the PRPP site of the enzyme (increasedKm PRPP, absence of product inhibition, absence of stabilization by PRPP) thein vivo activity in intact erythrocytes was negligible.
Organic acidaemia and hyperammonaemia: Review
Authors: Walser M. , Stewart P. M. 
Published: December 2005

AbstractRecent clinical and experimental evidence on the effects of organic acids in producing or ameliorating hyperammonaemia is reviewed. The importance of hepatic mitochondrialN‐acetylglutamate and its precursors, glutamate and acetyl‐CoA, in the control of ureagenesis and thus blood ammonia levels is emphasized by recent work. The hypothesis is proposed that protein loads stimulate urea cycle activity via glutamate‐induced changes inN‐acetylglutamate concentration, while the effects of organic acids on ureagenesis are related in a predictable way to their effects on hepatic concentrations of acetyl‐CoA.
Studies on human phenylalanine mono‐oxygenase. I. Restricted expression
Authors: Crawfurd M. A. , Gibbs D. A. , Sheppard D. M. 
Published: December 2005

AbstractEnzyme assays for phenylalanine mono‐oxygenase have been performed on a variety of non‐hepatic human cell types under varying conditions. The cell types studied were cultured fibroblasts, short‐term lymphocyte cultures, long‐term lymphoblastoid cultures, cultured amniotic fluid cells, hair roots and placental extracts. The cultured cells were assayed after growth under standard conditions and in the presence of a high concentration of substrate, a low concentration of end‐product, added hydrocortisone or dexamethasone and under combinations of these conditions. In no instance was a significant enzyme activity obtained.
S‐Adenosylhomocysteine hydrolase activity in a lymphoblastoid cell line from a patient with adenosine deaminase deficiency disease
Authors: Tsuchiya S. , Nakae S. , Konno T. , Tada K. 
Published: December 2005

AbstractS‐Adenosylhomocysteine (S‐AdoHcy) hydrolase activity in a lymphoblastoid cell line from a patient with adenosine deaminase deficiency disease (ADA(−)LCL) was found to be approximately 60% of that in ADA(+)lymphoblastoid cell lines.S‐AdoHcy hydrolase of ADA(−)LCL was more sensitive to inhibition by 2′‐deoxyadenosine as compared with that of ADA(+)LCL. The inhibitory effect of 2′‐deoxyadenosine was evident in cell growth and immunoglobulin production of lymphoblastoid cell lines.
Hypoxanthine‐guanine phosphoribosyltransferase variants: Correlation of clinical phenotype with enzyme activity
Authors: Page T. , Bakay B. , Nissinen E. , Nyhan W. L. 
Published: December 2005

AbstractA deficiency of the enzyme hypoxanthine‐guanine phosphoribosyltransferase (HGPRT) is associated with a varying clinical picture which may include hyperuricaemia, neurological abnormalities and bizarre self‐multilating behaviour. Due to technical problems with the usualin vitro enzyme assays, it has not been possible to establish a correlation between the degree of the enzyme deficiency and the severity of the clinical manifestations. In this study, the HGPRT activity of 12 patients with various clinical features was measured by quantitative analysis of the incorporation of radioactive precursors into purine compounds in intact fibroblasts. The results demonstrate that a correlation between the severity of the clinical symptoms and the degree of the enzyme deficiency as measured in intact fibroblasts does in fact exist.
Formiminoglutamic aciduria in a slightly retarded boy with chronic obstructive lung disease
Authors: Beck B. , Christensen E. , Brandt N. J. , Pedersen M. 
Published: December 2005

AbstractA 2‐year‐old boy excreted massive amounts of formiminoglutamic acid in urine. The substance was identified as authentic formiminoglutamic acid by two‐dimensional thin‐layer chromatography, column chromatography and enzymatic determination. After alkaline hydrolysis the substance was converted to glutamic acid. Serum amino acid concentrations were normal. The patient had normal serum and erythrocyte folate levels. The red blood cell picture was normal. The leukocytes showed slight hypersegmentation. From the age of 3 months he exhibited recurrent otitis media and severe pulmonary infections.He had a peculiar narrow‐headed look and marked universal hypotonia. The mental development was slightly retarded.Glutamate formiminotransferase deficiency is postulated. The findings lend support to the theory of glutamate formiminotransferase deficiency being a rather benign disorder of metabolism.
Aspartylglycosaminuria in an Italian family: Clinical and biochemical characteristics
Authors: Gehler J. , Sewell A. C. , Becker Christa , Spranger J. , Hartmann J. 
Published: December 2005

AbstractTwo members of a consanguineous Italian family are described with symptoms of aspartylglycosaminuria. Both patients exhibit mental retardation, some facial dysmorphism and discrete radiological abnormalities affecting the skull and vertebrae. Peripheral blood smears revealed multi‐vacuolated lymphocytes. Enzyme studies in leukocytes showed an absence of aspartylglucosaminidase activity. Urine analysis demonstrated abnormal oligosacchariduria. Angiokeratoma corporis diffusum was observed in one patient. The disease is seen as not being limited to Scandinavia or to patients of Scandinavian descent.
Substrates and fertilization for the rustic cultivation of in vitro propagated native orchids in Soconusco, Chiapas
Authors: Damon Anne , Soriano Marco Pérez , Rivera del Lourdes Maria 
Published: December 2005

AbstractNative orchid cultivation is a compatible alternative for impoverished coffee farmers in southeastern Mexico who are in crisis due to falling prices. Sustainable orchid cultivation is also an urgent necessity as an alternative to nonsustainable extraction from protected reserves, forest fragments and traditional coffee plantations, and to restore and conserve populations within these habitats. Our objective was to test the effects of locally available substrates and fertilizers upon orchids cultivated under typical rural conditions in coffee-producing areas in Soconusco, Chiapas. Seven species of epiphytic orchids native to Soconusco region—Cattleya aurantiaca, Brassavola nodosa, Prosthechea (Encyclia) chacaoensis, Anathallis (Pleurothallis) racemiflora, Cattleya skinneri, Cycnoches ventricosumandEncyclia cordigera—were propagatedin vitro, acclimatized and established in rustic orchid galleries in the home gardens and plantations of coffee growers. Locally available waste products were used as substrates: clay tiles, tree bark, bamboo, seed hulls of pataxte (Theobroma bicolor) and wire baskets filled with bark chips. Two cheap and readily available commercial foliar feeds, Algaenzims (an organic product) and Bayfolan (a synthetic product) were tested. First, the substrates alone were tested for a period of 6 months to 1 year, then a combination of substrates and fertilizers were tested for 6 months, for effects upon leaf and root growth and root number. The mortality rates of these nonsymbiotically propagated, epiphytic orchids during the acclimatization phase, prior to these experiments, were high, between 60 and 90%. Once established in rustic galleries, the young orchid plants showed no preference for a particular substrate, survival depended upon technical problems during establishment, relating to difficulties with the attachment of plants to substrates, and the variable quality of care and attention offered by the farmers. Both fertilizers significantly improved one or all the parameters studied, and possibly counteracted the negative effects of the absence of symbiotic fungi, which, under natural conditions, are essential for orchid seed germination and adequate development of the young plant. More than half of the producers did not continue with the orchid cultures for economic and cultural reasons.
An integrated approach to crop/livestock systems: Wintering beef cows on swathed crops
Authors: Karn J.F. , Tanaka D.L. , Liebig M.A. , Ries R.E. , Kronberg S.L. , Hanson J.D. 
Published: December 2005

AbstractGrain and livestock production have gradually been separated, as farmers have tended to specialize in one or the other. Some producers and scientists are beginning to question whether this is the best approach. An integrated crop/livestock project was initiated in 1999 to investigate potentially beneficial synergies to both enterprises. The cattle portion of this project involved wintering dry pregnant cows on swathed crop residue and drilled corn produced in a 3-year crop rotation, with all crops present each year. Swath grazing was initiated in mid-November of 1999, 2000 and 2001, and terminated in mid-February each year. Two replications of ten Hereford cows were rotationally grazed on swathed oat/pea and triticale crop residue and swathed drilled corn (RGSC). Comparison treatments were: cows grazing swathed western wheatgrass (SWWG) and cows fed hay in a drylot. Cows on the RGSC treatment were also fed a 20% crude protein supplement of oat/pea and triticale grain. Drilled corn was swathed in late September and western wheatgrass in early October. Three-year average winter-long weight gains for the RGSC cows were slightly lower (P<0.10) than those for drylot cows. Body condition scores generally followed the same pattern as weight gain data. Reproductive and calf performance data were not significantly different among wintering treatments. Average daily feed costs per cow over the three winters were US$0.49, 0.65 and 0.73 for RGSC, SWWG and baled hay in a drylot, respectively, providing a potential daily savings of US$0.24 per cow with the RGSC treatment compared to bale feeding in a drylot. Swath grazing did not appear to have any adverse affects on mid-aged beef cow performance and could potentially reduce winter feeding costs and manure handling problems. The main problem with swath grazing is potentially deep snow and icing of the swaths.
Comparison of consumer perceptions and preference toward organic versus conventionally produced foods: A review and update of the literature
Authors: Yiridoe Emmanuel K. , Bonti-Ankomah Samuel , Martin Ralph C. 
Published: December 2005

AbstractGrowing interest in organic agriculture has prompted numerous studies that compare various aspects of organic and conventionally produced foods. This paper provides a comprehensive evaluation of empirical studies comparing organic products and conventionally grown alternatives. The emphasis is on key organic consumer demand and marketing issues, including: (1) the implications of an economic definition of organically grown food for consumer demand; (2) attributes that shoppers consider most when comparing organic with conventionally grown products; (3) level and characteristics of consumer knowledge and awareness about organic food; (4) assessment methods and characteristics of organic consumer attitudes and preferences; (5) size of price premium and characteristics of consumers’ willingness-to-pay for organic products; and (6) profile of organic consumers. Overall, although there is some knowledge and awareness about organic products, consumers are not consistent in their interpretation of what is organic. Secondly, while consumers typically understand the broad issues about organic foods, many tend not to understand the complexities and niceties of organic farming practices and organic food quality attributes. Uncertainty regarding the true attributes of organic, and skepticism about organic labels, part of which stems from reported cases of (inadvertent) mislabeling, and product misrepresentation, and partly because of nonuniform organic standards and certification procedures, may hold some consumers back from purchasing organic. Thirdly, concern for human health and safety, which is a key factor that influences consumer preference for organic food, is consistent with observed deterioration in human health over time and, therefore, motivates consumers to buy organic food as insurance and/or investment in health. Fourthly, the proportion of consumers who are willing to pay a price premium for organic food decreases with premium level. On the other hand, premiums tend to increase with (combinations of) preferred attributes. In addition, demand tends to depend more on the price differential with respect to conventionally grown products, than on actual price. In contrast to sensitivity of demand to changes in price, income elasticity of demand for organic foods is generally small. Finally, it is important for policy analysts and researchers to note that organic fresh fruits and vegetables currently dominate the organic consumer's food basket. Furthermore, it is not clear whether frequent buyers consider particular organic products (e.g., organic meat) as normal goods, or if consumers consider such products as luxury goods.
Marketing locally produced foods: Consumer and farmer opinions in Washington County, Nebraska
Authors: Schneider Mindi L. , Francis Charles A. 
Published: December 2005

AbstractLocal food system potentials were studied in Washington County, Nebraska. As a departure from most studies of locally based systems, farmers were surveyed in addition to consumers for potential participation. Data about the current food system and opinions and preferences for local production, marketing, and purchasing of food were collected using self-administered mail questionnaires. The response rate was 35% for the farmer survey and 37% for the consumer survey. Results indicated that, on the farming side of the food system, conventional corn and soybean production and marketing predominated in Washington County, and farmer interest in producing for local markets was low. Consumers reported a high level of interest in purchasing food from farmers' markets, local grocery stores, local restaurants and directly from farms, and indicated a willingness to pay a price premium for local foods. They also reported that quality and taste were the most important factors in food purchase decisions, but environmentally friendly production and support for local farmers were also important. We conclude that there is tremendous potential for local marketing of farm products, but that there is a large gap between consumer demand and willingness of farmers to meet this demand. Further study of motivations and opinions of farmers is needed, in addition to determining production thresholds for the number of farms and farmers needed to meet local food demands.
A city under Siege: Banditry & modes of accumulation in Nairobi, 1991-2004
Authors: Katumanga Musambayi 
Published: December 2005

This is a study of the impact of political and economic liberalisation on modes of socio-economic engagement and accumulation in Kenya's capital city, Nairobi, subsequent to the introduction of multiparty ‘democracy’ in 1992.1 On the one hand economic liberalisation led to a diminished state-provisioning capacity and unwillingness to protect public interests. On the other hand, political conditionalities opened up political space but also spawned anomic tendencies within the regime and among social groups and individuals, with struggles in defence of economic position against each other at one level, and against the state and local councils at another. This account focuses on the political economy underlying the resultant urban banditry in Nairobi. It seeks to demonstrate how a besieged regime facilitates the criminalisation of urban existence in a bid to ensure its survival.
The argument here is that beleaguered regimes survive through a twin strategy. They privatise public violence and appropriate private violence. The net effect is the perversion of social order and the emergence of bandit economies. Regime longevity may derive not only from lack of an alternative leadership and organising ideology, but also from the threat to perceived benefits accruing from such informal economies. The ruling elite responds to the possibility of losing power by using neo-patrimonial structures to selectively allocate public spaces to their cronies, thereby subverting social order and undermining democratisation, security and social harmony; this in turn spawns urban banditry. Urban banditry here denotes the unregulated deployment of instruments of coercion by ruling elite and various elements within the citizenry in bids to facilitate acquisition of economic benefits and political leverage.
Abundance, morphological diversity, and spatial distribution of autotrophic picoplankton in Lake Hovsgol (Mongolia)
Authors: Belykh O. I. , Sorokovikova E. G. , Tikhonova I. V. , Fedotov A. F. 
Published: December 2005

Hovsgol is the largest deep lake in Central Asia located in the south-west of the Baikal Rift zone. Until now there has been only one study on the autotrophic picoplankton in Lake Hovsgol. The present work summarizes results of picoplankton studies obtained with light, epifluorescence and scanning electron microscopy. Microscopic analysis showed the presence of picoplankton cyanobacteria and algae in Lake Hovsgol. Autotrophic picoplankton of the lake is characterized by morphotype diversity of Synechococcus species, Synechocystis-like aggregates, and picocyanobacteria cells of small sizes. Picocyanobacteria are dominated by Synechococcus species with single, rarely double, coccoid cells. There is also a picoplankton form of the diatom Cyclotella ocellata reaches 4–13 percent of the total species abundance. Three picocyanobacterial cultures belonging to the genus Synechococcus and exhibiting distinct morphological and ultrastructural characteristics were isolated from the plankton samples.
Chlorophyll a content in the pelagic zone of Lake Hovsgol in August was 0.182–0.374 μ g l− 1. In February, the abundance of picoplankton cyanobacteria in the surface layer, which make up the basic autotrophic picoplankton mass, was 2.78 × 105 cells ml−1, in March 1.36 × 105 cells ml−1, and in July 6.06 × 104 cells ml−1. In August, picocyanobacteria abundance fell to 3.42 × 104 cells ml−1 in the 0 to 10 m layer and 7.52 × 104 cells ml−1 in the 0 to 50 m layer. Quantitative variations in autotrophic picoplankton development throughout the lake are negligible. The greatest abundance of picocyanobacteria is recorded in the hypolimnion, 25–50 m deep.
Gammarus aequicauda(Crustacea: Amphipoda): A potential test species in marine sediment toxicity assessment
Authors: Prato Ermelinda , Biandolino Francesca 
Published: December 2005

In this study, the amphipod Gammarus aequicauda was evaluated as a test organism for use in sediment toxicity bioassays. Sensitivity to noncontaminant variables, to the reference toxicants and to some contaminated field sediments was analysed. Amphipods were tolerant to various salinity and temperature combinations during a ten-day assay. The organisms tested with different type of diet showed highest survival on the natural diet.The organism density effect on survival and growth of Gammarus aequicauda during a 28-day assay was examined. The results indicated that the density did not affect survival and production, but the effect of density was significant on the average weight.No effect on survival was observed on three sediment types during the 10-day exposure. Sensitivity to contaminants was assessed using cadmium chloride and copper chloride as reference toxicants in a 96 h water-only test. Methods were developed for conducting a short-term toxicity test with cadmium chloride-spiked sediment using this species. Water-only testing revealed high sensitivity of amphipods to reference toxicants. Experiments conducted with organisms of four different size classes demonstrated no significant differences in sensitivity.