418 research outputs found

    The Federal Circuit: A Model for Reform?

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    Are our federal courts organized suitably to perform their mission of assuring coherent administration of our national law? Maybe not. The senior author of this Article, along with many others, argued to the contrary forty years ago. Now, experience with the United States Court of Appeals for the Federal Circuit tends to confirm that an alternative structure of the federal judiciary could better serve the need for coherent national law, and without serious adverse consequences. Perhaps, therefore, it is now time for Congress to reconsider the matter. We here suggest the possibility that the United States replicate the structure of the appellate courts of the Federal Republic of Germany, which, like the Federal Circuit, are specialized to assure coherent and consistent interpretation of that nation’s laws. Advances in technology have greatly reduced the need for the traditional regionalization of the federal appellate process, so that the model supplied by the Federal Circuit may offer new hope that our national law could be administered with substantially greater coherence and efficiency than the present system of conflicted circuits allows

    Progress in Establishing Common Standards for Exchanging Proteomics Data: The Second Meeting of the HUPO Proteomics Standards Initiative

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    The Proteomics Standards Initiative (PSI) aims to define community standards for data representation in proteomics and to facilitate data comparison, exchange and verification. Rapid progress has been made in the development of common standards for data exchange in the fields of both mass spectrometry and protein–protein interactions since the first PSI meeting [1]. Both hardware and software manufacturers have agreed to work to ensure that a proteomics-specific extension is created for the emerging ASTM mass spectrometry standard and the data model for a proteomics experiment has advanced significantly. The Protein–Protein Interactions (PPI) group expects to publish the Level 1 PSI data exchange format for protein–protein interactions by early summer this year, and discussion as to the additional content of Level 2 has been initiated

    The HUPO Proteomics Standards Initiative Meeting: Towards Common Standards for Exchanging Proteomics Data

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    The Proteomics Standards Initiative (PSI) aims to define community standards for data representation in proteomics and to facilitate data comparison, exchange and verification. Initially the fields of protein–protein interactions (PPI) and mass spectroscopy have been targeted and the inaugural meeting of the PSI addressed the questions of data storage and exchange in both of these areas. The PPI group rapidly reached consensus as to the minimum requirements for a data exchange model; an XML draft is now being produced. The mass spectroscopy group have achieved major advances in the definition of a required data model and working groups are currently taking these discussions further. A further meeting is planned in January 2003 to advance both these projects

    Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H.

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    Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs). Historically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegeneration with death in the first decade due to cardio-pulmonary complications. New data suggest that inflammation may play a role in MPS pathophysiology. To date there is almost no information on the pathophysiologic changes within the cerebral spinal fluid (CSF) of these patients. We evaluated the CSF of 25 consecutive patients with MPS-IH. While CSF glucose and total protein were within the normal range, we found a significantly mean elevated CSF opening pressure at 24 cm H2O (range 14-37 cm H2O). We observed a 3-fold elevation in CSF heparan sulfate and a 3-8 fold increase in MPS-IH specific non-reducing ends, I0S0 and I0S6. Cytokine analyses in CSF of children with MPS-IH showed significantly elevated inflammatory markers including: MCP-1 SDF-1a, IL-Ra, MIP-1b, IL-8, and VEGF in comparison to unaffected children. This is the largest report of CSF characteristics in children with MPS-IH. Identification of key biomarkers may provide further insight into the inflammatory-mediated mechanisms related to MPS diseases and perhaps lead to improved targeted therapies

    Biochemical and clinical response after umbilical cord blood transplant in a boy with early childhood-onset beta-mannosidosis.

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    BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available. METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease. RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of the brain remained stable. Neurocognitive evaluation revealed test point gains, although the magnitude of improvement was less than expected for age, causing lower IQ scores that represent a wider developmental gap between the patient and unaffected peers. CONCLUSION: Our findings suggest that hematopoietic cell transplant can correct the biochemical defect in β-mannosidosis, although preservation of the neurocognitive trajectory may be a challenge

    Microbial Activity of Soil Following Steam Treatment

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    The effect of steam treatment on subsurface aerobic and anaerobic microbial communities was investigated using multiple microbial assays. Soil samples were gathered and analyzed prior to, one month after, and eight months after a five-month field pilot test of steam injection and extraction. Aerobic soil samples were analyzed by respirometry, plate counts, and direct microscopic counts. Anaerobic microbial activity was examined by monitoring methane generation in anaerobic microcosms with gas chromatography. Respirometry showed pre-steam CO2 production was consistent with natural attenuation, post-steam (one month) CO2 production was below detection, and post-steam (eight months) CO2 production was about half of pre-steam. Post-steam (one and eight month) plate counts were one to four orders of magnitude lower than the pre-steam samples. Direct microscopic counts showed post-steam (one and eight month) cell numbers were higher than the pre-steam counts, but based on plate counts these cells were mostly non-viable. Significant amounts of methane and hydrogen were generated from pre-steam anaerobic microcosms, but post-steam microcosms had no detectable methane, and only trace amounts of hydrogen. Terminal restriction fragment (TRF) analysis was performed to determine the diversity of the microbial community before and after steam treatment. Pre-steam TRF analysis showed distinct differences in the microbial communities above and below the smear zone. Post-steam TRF analyses were not possible because insufficient DNA could be extracted from the soil

    Hydrogeological challenges in a low carbon economy

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    Hydrogeology has traditionally been regarded as the province of the water industry, but it is increasingly finding novel applications in the energy sector. Hydrogeology has a longstanding role in geothermal energy exploration and management. Although aquifer management methods can be directly applied to most high-enthalpy geothermal reservoirs, hydrogeochemical inference techniques differ somewhat owing to peculiarities of high-temperature processes. Hydrogeological involvement in the development of ground-coupled heating and cooling systems using heat pumps has led to the emergence of the sub-discipline now known as thermogeology. The patterns of groundwater flow and heat transport are closely analogous and can thus be analysed using very similar techniques. Without resort to heat pumps, groundwater is increasingly being pumped to provide cooling for large buildings; the renewability of such systems relies on accurate prediction and management of thermal breakthrough from reinjection to production boreholes. Hydrogeological analysis can contribute to quantification of accidental carbon emissions arising from disturbance of groundwater-fed peatland ecosystems during wind farm construction. Beyond renewables, key applications of hydrogeology are to be found in the nuclear sector, and in the sunrise industries of unconventional gas and carbon capture and storage, with high temperatures attained during underground coal gasification requiring geothermal technology transfer
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