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In order to step up its efforts in reducing climate change, the European Commission (hereafter: the Commission) has launched in June 2000 its European climate change program (hereafter: ECCP). This wide-ranging stakeholder consultation aimed at identifying and developing all elements necessary for a European climate change strategy. The ECCP formally came to a close in April 2003. 相似文献
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Antibody Epitope of Human α‐Galactosidase A Revealed by Affinity Mass Spectrometry: A Basis for Reversing Immunoreactivity in Enzyme Replacement Therapy of Fabry Disease
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Dr. Zdenek Kukacka Dr. Marius Iurascu Loredana Lupu Hendrik Rusche Dr. Mary Murphy Lorenzo Altamore Fabio Borri Dr. Stefan Maeser Prof. Dr. Anna Maria Papini Prof. Dr. Julia Hennermann Prof. Dr. Michael Przybylski 《ChemMedChem》2018,13(9):909-915
α‐Galactosidase (αGal) is a lysosomal enzyme that hydrolyses the terminal α‐galactosyl moiety from glycosphingolipids. Mutations in the encoding genes for αGal lead to defective or misfolded enzyme, which results in substrate accumulation and subsequent organ dysfunction. The metabolic disease caused by a deficiency of human α‐galactosidase A is known as Fabry disease or Fabry–Anderson disease, and it belongs to a larger group known as lysosomal storage diseases. An effective treatment for Fabry disease has been developed by enzyme replacement therapy (ERT), which involves infusions of purified recombinant enzyme in order to increase enzyme levels and decrease the amounts of accumulated substrate. However, immunoreactivity and IgG antibody formation are major, therapy‐limiting, and eventually life‐threatening complications of ERT. The present study focused on the epitope determination of human α‐galactosidase A against its antibody formed. Here we report the identification of the epitope of human αGal(309–332) recognized by a human monoclonal anti‐αGal antibody, using a combination of proteolytic excision of the immobilized immune complex and surface plasmon resonance biosensing mass spectrometry. The epitope peptide, αGal(309–332), was synthesized by solid‐phase peptide synthesis. Determination of its affinity by surface plasmon resonance analysis revealed a high binding affinity for the antibody (KD=39×10?9 m ), which is nearly identical to that of the full‐length enzyme (KD=16×10?9 m ). The proteolytic excision affinity mass spectrometry method is shown here to be an efficient tool for epitope identification of an immunogenic lysosomal enzyme. Because the full‐length αGal and the antibody epitope showed similar binding affinities, this provides a basis for reversing immunogenicity upon ERT by: 1) treatment of patients with the epitope peptide to neutralize antibodies, or 2) removal of antibodies by apheresis, and thus significantly improving the response to ERT. 相似文献
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Jensen J. Eric; Daniels Melissa; Haws Charlotte; Bolo Nicolas R.; Lyoo In Kyoon; Yoon Sujung J.; Cohen Bruce M.; Stoll Andrew L.; Rusche James R.; Renshaw Perry F. 《Canadian Metallurgical Quarterly》2008,16(3):199
Eleven patients with bipolar depression were given doses of up to 18 g per day of triacetyluridine (TAU) over 6 weeks to test the effect of uridine on symptoms of depression via Montgomery-Asberg Depression Rating Scale (MADRS; Asberg, Montgomery, Perris, Schalling, & Sedvall, 1978) scores and on cellular bioenergetics using phosphorus magnetic resonance spectroscopic imaging (31P-MRSI). All patients and comparison participants (n = 9) completed baseline 31P-MRSI scans, and 9 patients completed posttherapy scans. The percentage changes for MADRS scores (Week 2, -23.8; Week 3, -34.9; Week 4, -42.5) and the time effects of TAU on MADRS scores (Week 2, z = -2.07, p = .039; Week 3, z = -4.28, p 相似文献
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Wilhelm Rusche 《变频器世界》2009,(7):101-102,81
可靠性与能效是当今逆变器设计考虑的两个主要因素。英飞凌全新的EconoPACKTM4将坚固的模块设计与全新高能效IGBT4和EmitterControlled4二极管技术融合在一起。 相似文献
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Ernst Schmitz Roland Ohme Siegfried Schramm Helmut Striegler Hans-Ulrich Heyne Jochen Rusche 《Advanced Synthesis \u0026amp; Catalysis》1977,319(2):195-200
Efforts into a Novel Caprolactame Synthesis. I. Preparation of 3,3-Pentamethylene Oxaziridine from Cyclohexanone and Chloramine 3,3-Pentamethylene-oxaziridine 5 , an isomer of cyclohexanone oxime and a potential precursor of caprolactame, is prepared from cyclohexanone, ammonia, and hypochlorite in 73 percent yield, from cyclohexanone and chloramine solutions in 85 percent yield. A crystalline 1:1 adduct of chloramine to cyclohexanone can be cyclized to 5 in 91 percent yield. 相似文献
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Deposition of HWCVD poly-Si films at a high growth rate 总被引:2,自引:0,他引:2
J. K. Rath A. J. Hardeman C. H. M. van der Werf P. A. T. T. van Veenendaal M. Y. S. Rusche R. E. I. Schropp 《Thin solid films》2003,430(1-2):67-72
The process parameters for high growth rate poly-silicon films by hot-wire chemical vapour deposition have been explored. A four-wire hot wire assembly has been employed for this purpose. High silane to hydrogen flow ratios and high wire temperatures are the key process parameters to achieve high growth rate and growth rates higher than 5 nm/s can be achieved. The process conditions to incorporate high hydrogen content into the material for passivation of defects and donor states have been identified as high hydrogen dilution and lower wire temperature. With these procedures poly-Si films deposited at 1.3 nm/s showed high ambipolar diffusion length of 132 nm. Incorporating such poly-Si films as i-layer, n–i–p solar cell on stainless steel substrate without back reflector showed an efficiency of 4.4%. 相似文献