Fanconi-Bickel syndrome and autosomal recessive proximal tubulopathy with hypercalciuria (ARPTH) are allelic variants caused by GLUT2 mutations.

CONTEXT: Many inherited disorders of calcium and phosphate homeostasis are unexplained at the molecular level. OBJECTIVE: The objective of the study was to identify the molecular basis of phosphate and calcium abnormalities in two unrelated, consanguineous families. PATIENTS: The affected members in family 1 presented with rickets due to profound urinary phosphate-wasting and hypophosphatemic rickets. In the previously reported family 2, patients presented with proximal renal tubulopathy and hypercalciuria yet normal or only mildly increased urinary phosphate excretion. METHODS: Genome-wide linkage scans and direct nucleotide sequence analyses of candidate genes were performed. Transport of glucose and phosphate by glucose transporter 2 (GLUT2) was assessed using Xenopus oocytes. Renal sodium-phosphate cotransporter 2a and 2c (Npt2a and Npt2c) expressions were evaluated in transgenically rescued Glut2-null mice (tgGlut2-/-). RESULTS: In both families, genetic mapping and sequence analysis of candidate genes led to the identification of two novel homozygous mutations (IVS4-2A>G and R124S, respectively) in GLUT2, the gene mutated in Fanconi-Bickel syndrome, a rare disease usually characterized by renal tubulopathy, impaired glucose homeostasis, and hepatomegaly. Xenopus oocytes expressing the [R124S]GLUT2 mutant showed a significant reduction in glucose transport, but neither wild-type nor mutant GLUT2 facilitated phosphate import or export; tgGlut2-/- mice demonstrated a profound reduction of Npt2c expression in the proximal renal tubules. CONCLUSIONS: Homozygous mutations in the facilitative glucose transporter GLUT2, which cause Fanconi-Bickel syndrome, can lead to very different clinical and biochemical findings that are not limited to mild proximal renal tubulopathy but can include significant hypercalciuria and highly variable degrees of urinary phosphate-wasting and hypophosphatemia, possibly because of the impaired proximal tubular expression of Npt2c.

Trade liberalization in Uruguay: Domestic and foreign firms

The reallocation of resources is one of the main impacts of tradeliberalization processes. In the case of manufacturing industries resourceswill be reallocated from import--competing sectors to export--orientedsectors. This paper studies the effects that a more open economic environmenthas had on the entry conditions for foreign and domestic firms in Uruguayanmanufacturing industries. We find significant differences in the behaviorof foreign and domestic firms, both when they are incumbents or when theyact as potential entrants. In general, foreign firms seem to be moresuccessful in applying entry deterring strategies, due to advantages inforeign markets, deeper financial resources or better technological capabilities.They also appear to be more responsive to entry conditions when theyface the prospects of entering a given industry.

A T42A Ran Mutation: Differential Interactions with Effectors and Regulators, and Defect in Nuclear Protein Import

Ran, the small, predominantly nuclear GTPase, has been implicated in the regulation of a variety of cellular processes including cell cycle progression, nuclear-cytoplasmic trafficking of RNA and protein, nuclear structure, and DNA synthesis. It is not known whether Ran functions directly in each process or whether many of its roles may be secondary to a direct role in only one, for example, nuclear protein import. To identify biochemical links between Ran and its functional target(s), we have generated and examined the properties of a putative Ran effector mutation, T42A-Ran. T42A-Ran binds guanine nucleotides as well as wild-type Ran and responds as well as wild-type Ran to GTP or GDP exchange stimulated by the Ran-specific guanine nucleotide exchange factor, RCC1. T42A-Ran·GDP also retains the ability to bind p10/NTF2, a component of the nuclear import pathway. In contrast to wild-type Ran, T42A-Ran·GTP binds very weakly or not detectably to three proposed Ran effectors, Ran-binding protein 1 (RanBP1), Ran-binding protein 2 (RanBP2, a nucleoporin), and karyopherin ß (a component of the nuclear protein import pathway), and is not stimulated to hydrolyze bound GTP by Ran GTPase-activating protein, RanGAP1. Also in contrast to wild-type Ran, T42A-Ran does not stimulate nuclear protein import in a digitonin permeabilized cell assay and also inhibits wild-type Ran function in this system. However, the T42A mutation does not block the docking of karyophilic substrates at the nuclear pore. These properties of T42A-Ran are consistent with its classification as an effector mutant and define the exposed region of Ran containing the mutation as a probable effector loop.

Macronutrient uptake, accumulation and export by the irrigated 'vitória' pineapple plant

The nutritional state of the pineapple plant has a large effect on plant growth, on fruit production, and fruit quality. The aim of this study was to assess the uptake, accumulation, and export of nutrients by the irrigated 'Vitória' pineapple plant during and at the end of its development. A randomized block statistical design with four replications was used. The treatments were defined by different times of plant collection: at 270, 330, 390, 450, 510, 570, 690, 750, and 810 days after planting (DAP). The collected plants were separated into the following components: leaves, stem, roots, fruit, and slips for determination of fresh and dry matter weight at 65 ºC. After drying, the plant components were ground for characterization of the composition and content of nutrients taken up and exported by the pineapple plant. The results were subjected to analysis of variance, and non-linear regression models were fitted for the significant differences identified by the F test (p<0.01). The leaves and the stem were the plant components that showed the greatest accumulation of nutrients. For production of 72 t ha-1 of fruit, the macronutrient accumulation in the 'Vitória' pineapple exhibited the following decreasing order: K > N > S > Ca > Mg > P, which corresponded to 898, 452, 134, 129, 126, and 107 kg ha-1, respectively, of total accumulation. The export of macronutrients by the pineapple fruit was in the following decreasing order: K > N > S > Ca > P > Mg, which was equivalent to 18, 17, 11, 8, 8, and 5 %, respectively, of the total accumulated by the pineapple. The 'Vitória' pineapple plant exported 78 kg ha-1 of N, 8 kg ha-1 of P, 164 kg ha-1 of K, 14 kg ha-1 of S, 10 kg ha-1 of Ca, and 6 kg ha-1 of Mg by the fruit. The nutrient content exported by the fruits represent important components of nutrient extraction from the soil, which need to be restored, while the nutrients contained in the leaves, stems and roots can be incorporated in the soil within a program of recycling of crop residues.

Effect of potassium sources and rates on arabica coffee yield, nutrition, and macronutrient export

The use of potassium (K) rock powder can be an alternative for K supply of crops. Thus, to reduce K fertilizer imports from abroad, possibilities of extracting this nutrient from Brazilian rocks are being studied. The objective was to evaluate the effect of phonolite rock powder (F2) as K source (Ekosil®) on the air-dried fruit yield, nutrition and macronutrient export of Arabica coffee. The experiment was carried out on a dystroferric Red Latosol (Typic Haplorthox), in Piraju, São Paulo State, Brazil, in the 2008/09 and 2009/10 growing seasons. The experimental design was a randomized complete block, in a factorial 2 × 3 + 1 arrangement, with four replications. The treatments consisted of two K sources (KCl - 58 % of K2O and F2 - 8.42 % K2O) and three rates ½-, 1-, and 2-fold the K2O rate recommended for coffee, i.e., 75, 150, and 300 kg ha-1 of K2O), plus a control (without K application). Potassium supply increased coffee yield, regardless of the source. Application of source F2 increased coffee yield similarly to KCl at the recommended K rate for coffee (150 kg ha-1 K2O), proving efficient as K supply for coffee. Potassium application increased macronutrient export in coffee, especially in the growing season with higher yield.

Export of dissolved organic carbon, nitrogen and phosphorus following clear-cutting of three Norway spruce forest growing on drained peatlands in southern Finland

Abstract

Functional expression of PHO1 to the Golgi and trans-Golgi network and its role in export of inorganic phosphate.

Arabidopsis thaliana PHO1 is primarily expressed in the root vascular cylinder and is involved in the transfer of inorganic phosphate (Pi) from roots to shoots. To analyze the role of PHO1 in transport of Pi, we have generated transgenic plants expressing PHO1 in ectopic A. thaliana tissues using an estradiol-inducible promoter. Leaves treated with estradiol showed strong PHO1 expression, leading to detectable accumulation of PHO1 protein. Estradiol-mediated induction of PHO1 in leaves from soil-grown plants, in leaves and roots of plants grown in liquid culture, or in leaf mesophyll protoplasts, was all accompanied by the specific release of Pi to the extracellular medium as early as 2-3 h after addition of estradiol. Net Pi export triggered by PHO1 induction was enhanced by high extracellular Pi and weakly inhibited by the proton-ionophore carbonyl cyanide m-chlorophenylhydrazone. Expression of a PHO1-GFP construct complementing the pho1 mutant revealed GFP expression in punctate structures in the pericycle cells but no fluorescence at the plasma membrane. When expressed in onion epidermal cells or in tobacco mesophyll cells, PHO1-GFP was associated with similar punctate structures that co-localized with the Golgi/trans-Golgi network and uncharacterized vesicles. However, PHO1-GFP could be partially relocated to the plasma membrane in leaves infiltrated with a high-phosphate solution. Together, these results show that PHO1 can trigger Pi export in ectopic plant cells, strongly indicating that PHO1 is itself a Pi exporter. Interestingly, PHO1-mediated Pi export was associated with its localization to the Golgi and trans-Golgi networks, revealing a role for these organelles in Pi transport.

Solid and liquid biofuels markets in Finland - a study on international biofuels trade

This study considered the current situation of solid and liquid biofuels markets and international biofuels trade in Finland and identified the challenges ofthe emerging international biofuels markets for Finland. The fact that industryconsumes more than half of the total primary energy, widely applied combined heat and power production (CHP) and a high share of biofuels in the total energy consumption are specific to the Finnish energy system. One third of the electricity is generated in CHP plants. As much as 27% of the total energy consumption ismet by using wood and peat, which makes Finland the leading country in the use of biofuels. Finland has made a commitment to maintain greenhouse gas emissions at the 1990 level at the highest during the period 2008-2012. The Finnish energypolicy aims to achieve the target, and a variety of measures are taken to promote the use of renewable energy sources and especially wood fuels. In this study, the wooden raw material streams of the forest industry were included the international biofuels trade in addition to biomass streams that are traded for energy production. In 2004, as much as 45% of the raw wood importedinto Finland ended up in energy production. The total international trading of biofuels was evaluated at 72 PJ, of which the majority, 58 PJ, was raw wood. About 22% of wood based energy in Finland originated from imported raw wood. Tall oil and wood pellets composed the largest export streams of biofuels. The annual turnover of international biofuels trade was estimated at about ¤ 90 million fordirect trade and at about ¤ 190 million for indirect trade. The forest industryas the biggest user of wood, and the producer and user of wood fuels has a central position in biomass and biofuels markets in Finland. Lately, the international aspects of Finnish biofuels markets have been emphasised as the import of rawwood and the export of wood pellets have increased. Expanding the use of biofuels in the road transportation sector would increase the international streams ofbiofuels in Finland. In coming years, the international trading of biomass for energy purposes can be expected to continue growing.

MetaNetX/MNXref - reconciliation of metabolites and biochemical reactions to bring together genome-scale metabolic networks.

MetaNetX is a repository of genome-scale metabolic networks (GSMNs) and biochemical pathways from a number of major resources imported into a common namespace of chemical compounds, reactions, cellular compartments-namely MNXref-and proteins. The MetaNetX.org website (http://www.metanetx.org/) provides access to these integrated data as well as a variety of tools that allow users to import their own GSMNs, map them to the MNXref reconciliation, and manipulate, compare, analyze, simulate (using flux balance analysis) and export the resulting GSMNs. MNXref and MetaNetX are regularly updated and freely available.

SMRT-mediated co-shuttling enables export of class IIa HDACs independent of their CaM kinase phosphorylation sites

The Class IIa histone deacetylases (HDAC)4 and HDAC5 play a role in neuronal survival and behavioral adaptation in the CNS. Phosphorylation at 2/3 N-terminal sites promote their nuclear export. We investigated whether non-canonical signaling routes to Class IIa HDAC export exist because of their association with the co-repressor Silencing Mediator Of Retinoic And Thyroid Hormone Receptors (SMRT). We found that, while HDAC5 and HDAC4 mutants lacking their N-terminal phosphorylation sites (HDAC4(MUT), HDAC5(MUT)) are constitutively nuclear, co-expression with SMRT renders them exportable by signals that trigger SMRT export, such as synaptic activity, HDAC inhibition, and Brain Derived Neurotrophic Factor (BDNF) signaling. We found that SMRT's repression domain 3 (RD3) is critical for co-shuttling of HDAC5(MUT), consistent with the role for this domain in Class IIa HDAC association. In the context of BDNF signaling, we found that HDAC5(WT), which was more cytoplasmic than HDAC5(MUT), accumulated in the nucleus after BDNF treatment. However, co-expression of SMRT blocked BDNF-induced HDAC5(WT) import in a RD3-dependent manner. In effect, SMRT-mediated HDAC5(WT) export was opposing the BDNF-induced HDAC5 nuclear accumulation observed in SMRT's absence. Thus, SMRT's presence may render Class IIa HDACs exportable by a wider range of signals than those which simply

IEA Bioenergy Task 40 “Sustainable International Bioenergy Trade: Securing supply and demand” Country report of Finland 2008

This study considered the current situation of biofuels markets in Finland. The fact that industry consumes more than half of the total primary energy, widely applied combined heat and power production and a high share of solid biomass fuels in the total energy consumption are specific to the Finnish energy system. Wood is the most important source of bioenergy in Finland, representing 21% of the total energy consumption in 2006. Almost 80% of the wood-based energy is recovered from industrial by-products and residues. Finland has commitment itself to maintaining its greenhouse gas emissions at the 1990 level, at the highest, during the period 2008–2012. The energy and climate policy carried out in recent years has been based on the National Energy and Climate introduced in 2005. The Finnish energy policy aims to achieve the target, and a variety of measures are taken to promote the use of renewable energy sources and especially wood fuels. In 2007, the government started to prepare a new long-term (up to the year 2050) climate and energy strategy that will meet EU’s new targets for the reduction of green house gas emissions and the promotion of renewable energy sources. The new strategy will be introduced during 2008. The international biofuels trade has a substantial importance for the utilisation of bioenergy in Finland. In 2006, the total international trading of solid and liquid biofuels was approximately 64 PJ of which import was 61 PJ. Most of the import is indirect and takes place within the forest industry’s raw wood imports. In 2006, as much as 24% of wood energy was based on foreignorigin wood. Wood pellets and tall oil form the majority of export streams of biofuels. The indirect import of wood fuels increased almost 10% in 2004–2006, while the direct trade of solid and liquid biofuels has been almost constant.

Market of biomass fuels in Finland : IEA Bioenergy Task 40 and EUBIONET III - Country report of Finland 2009

This study considered the current situation of solid and liquid biomass fuels in Finland. The fact that industry consumes more than half of the total primary energy, widely applied combined heat and power production and a high share of solid biomass fuels in the total energy consumption are specific to the Finnish energy system. Wood is the most important source of bioenergy in Finland, representing 20% of the total energy consumption in 2007. Almost 80% of the woodbased energy is recovered from industrial by-products and residues. As a member of the European Union, Finland has committed itself to the Union’s climate and energy targets, such as reducing its overall emissions of green house gases to at least 20% below 1990 levels by 2020, and increasing the share of renewable energy in the gross final consumption. The renewable energy target approved for Finland is 38%. The present National Climate and Energy Strategy was introduced in November 2008. The strategy covers climate and energy policy measures up to 2020, and in brief thereafter, up to 2050. In recent years, the actual emissions have exceeded the Kyoto commitment and the trend of emissions is on the increase. In 2007, the share of renewable energy in the gross final energy consumption was approximately 25% (360 PJ). Without new energy policy measures, the final consumption of renewable energy would increase to 380 PJ, which would be approximately only 31% of the final energy consumption. In addition, green house gas emissions would exceed the 1990 levels by 20%. Meeting the targets will need the adoption of more active energy policy measures in coming years. The international trade of biomass fuels has a substantial importance for the utilisation of bioenergy in Finland. In 2007, the total international trading of solid and liquid biomass fuels was approximately 77 PJ, of which import was 62 PJ. Most of the import is indirect and takes place within the forest industry’s raw wood imports. In 2007, as much as 21% of wood energy was based on foreign-origin wood. Wood pellets and tall oil form the majority of export streams of biomass fuels. The indirect import of wood fuels peaked in 2006 to 61 PJ. The foreseeable decline in raw wood import to Finland will decrease the indirect import of wood fuels. In 2004– 2007, the direct trade of solid and liquid biomass fuels has been on a moderate growth path. In 2007, the import of palm oil and export of bio-diesel emerged, as a large, 170 000 t/yr biodiesel plant came into operation in Porvoo.

Buying behavior of public organizations and its influence on export partner governance

Public organizations form a significant part of any economy, yet their buying behavior has received very little attention. Operating with complex public sector is further complicated when sales and marketing activities are done by foreign export partners. This thesis explores the buying behavior of public organizations and how partnership governance is influenced by it. The theoretical part focuses on two subjects. Firstly the strictly regulated purchase process, rigid decision making and other special characteristics of public organizations are examined. Secondly the thesis examines partnership governance forms and how coordination is arranged in the relationships. The empirical part investigates the subjects of public organization buying behavior and partnership governance using a case study of a Finnish SME and their two export partners. The findings suggest high degree of uniformity between public organizations enables the use of unilateral governance forms. By creating mutual dependence through training the possibility of adopting a purely bilateral governance form exists as well.