Sanfilippo D syndrome: Correction of glucosamine-6-sulphatase deficiency following fibroblast culture in chang's media

The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-³H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.     

Antenatal diagnosis of a de novo paracentric inversion of chromosome 11

A de novo paracentric inversion of chromosome 11 identified through antenatal diagnosis is described along with long-term follow-up information on the child and discussion of reported experiences with de novo inversions.     

e-Serem – A Web-Based Manual For The Estimation of Emission Reductions From JI and CDM Projects

This paper presentse-SEREM (Smart Emission ReductionEstimation Manual), a cost-free, easilyaccessed and updated, web-based manual forestimating emission reductions from Joint Implementation (JI) andClean Development Mechanism (CDM) projects. e-SEREM's main functions arethe selection of a benchmark for a specificproject type in the power or heat sectorand the calculation of the annual andcumulative emission reductions accrued bythis project for its crediting lifetime.e-SEREM was developed in order to test itsapplicability and practicality in assistingproject developers and evaluators toelaborate baselines easily and calculatethe emission credits earned by candidate JIor CDM projects hosted in several countries.     

Particle monitoring techniques for water treatment applications

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Industrial ecology: a new field or only a metaphor?

In the 10 years since industrial ecology first became a topic of academic interest, it has grown as a field of inquiry and has produced a community of practice in several sectors including academia, business, and government. Even as the shape of industrial ecology becomes clearer, questions remain as to its lasting power beyond the metaphor that gave it its distinctiveness. This paper examines the development of industrial ecology and assesses its progress towards becoming a field of academic inquiry. And, in a related analysis, I look at the progress industrial ecology has made in establishing itself as an institutional (cultural) basis for action in the above sectors. Ideas like industrial ecology must become institutionalized if they are to have much effect on the reality of everyday activities.     

CLIMATE CHANGE AND NATURAL HAZARDS IN NORTHERN CANADA: INTEGRATING INDIGENOUS PERSPECTIVES WITH GOVERNMENT POLICY

A study of the relationship between natural hazards and climate change in the international context provides the background for a discussion of the expected changes. In the context of this global discussion, this paper reviews the current perspectives of those natural hazards that are likely to be influenced by climate change, using northern Canada as a regional case study. The northern implications of the United Nations Framework Convention on Climate Change are examined, including the status of climate change action by the northern territorial governments, the evolving role of indigenous people, and the responsibility for climate change impacts. The difficulties surrounding natural hazards research in remote locations, and the approaches of indigenous people to natural hazards are then presented. The paper concludes with a suggested policy approach for climate change and natural hazards in northern Canada, underscoring the need for more comprehensive adaptive strategies to complement the current tendency to focus on the mitigation of greenhouse gases produced in this region.     

International competitive displacement from environmental control The quantitative gains from methodological refinement

This paper demonstrates empirically the gain from adopting more general equilibrium approaches in projecting the effects on domestic output and international trade of environmental controls. The suggested models include a partial equilibrium case where all industries are treated independently, an intermediate case incorporating effects in supplying industries, and general equilibrium formulations based on alternative macroeconomic conditions. U. S. data is used to evaluate these models. Under both pollutor pays and subsidy financing schemes, moving from the simplest case to the intermediate model results in quite different industry projections. Including macroeconomic factors appears particularly important in the classical setting.     

Oil/Suspended Particulate Material Interactions and Sedimentation

The interactions of physically dispersed oil droplets with suspended particulate material (SPM) can be important for the transport of bulk quantities of spilled crude oil and polycyclic aromatic hydrocarbons (PAH) to subtidal sediments. The literature regarding oil/SPM interactions is reviewed, and results from whole-oil droplet/SPM interaction kinetics and pure-component (Prudhoe Bay crude oil distillate cut) equilibrium partitioning experiments are presented. The effects of oil type, SPM characteristics, and salinity on the interaction rates are examined, and the importance of whole-oil droplet/SPM interactions on particle agglomeration and settling behavior are discussed. Whole-oil droplet/SPM interactions are retarded as oil droplet dispersion into the water column is inhibited by oil viscosity increases due to evaporation weathering and water-in-oil emulsification. Compared to whole oil droplet/SPM interactions, dissolved-component/SPM adsorption is not as significant for transport of individual components to sediments. The information presented in this paper can be used to augment computer-based models designed to predict oil-spill trajectories, oil-weathering behavior, and spilled oil impacts to the marine environment.