Superconductivity for Large Scale Wind Turbines

A conceptual design has been completed for a 10MW superconducting direct drive wind turbine generator employing low temperature superconductors for the field winding. Key technology building blocks from the GE Wind and GE Healthcare businesses have been transferred across to the design of this concept machine. Wherever possible, conventional technology and production techniques have been used in order to support the case for commercialization of such a machine. Appendices A and B provide further details of the layout of the machine and the complete specification table for the concept design. Phase 1 of the program has allowed us to understand the trade-offs between the various sub-systems of such a generator and its integration with a wind turbine. A Failure Modes and Effects Analysis (FMEA) and a Technology Readiness Level (TRL) analysis have been completed resulting in the identification of high risk components within the design. The design has been analyzed from a commercial and economic point of view and Cost of Energy (COE) calculations have been carried out with the potential to reduce COE by up to 18% when compared with a permanent magnet direct drive 5MW baseline machine, resulting in a potential COE of 0.075 $/kWh. Finally, a top-level commercialization plan has been proposed to enable this technology to be transitioned to full volume production. The main body of this report will present the design processes employed and the main findings and conclusions

Long-term course of restless legs syndrome in dialysis patients after kidney transplantation

Restless legs syndrome (RLS) is a common cause of sleep disturbance and is frequently experienced by hemodialysis patients. Factors triggering the disease in uremia have not yet been identified. To our knowledge, the course of RLS symptoms after kidney transplantation has not been investigated systematically. We investigated the clinical long-term course of RLS in hemodialysis patients who underwent kidney transplantation. Patients were given a standardized questionnaire three times: at baseline, and twice after their kidney transplants. The severity of RLS was rated by the patients (0 = no symptoms, 10 = very severe symptoms). The description of the final outcome was based on the last follow- up visit. Eleven of 64 hemodialysis patients with RLS received a transplant (5 men, 6 women; severity of RLS at baseline, 7.8 +/- 0.7 [mean +/- SEM]). In all patients, RLS symptoms disappeared within 1 to 21 days after transplantation. At follow-up visits, 4 patients whose transplanted kidneys still functioned well were still free of RLS symptoms up to the longest follow-up period of 9 years. In 3 other patients, RLS symptoms gradually reappeared (severity, 1 +/- 0). In 3 of 11 patients, the transplant failed and RLS symptoms reoccurred within10 days to 2 months (severity, 7.3 +/- 2.6). RLS symptoms reoccurred in 1 patient with failure of the transplant but disappeared again after a second, successful transplant. Kidney transplantation has a strong and positive influence on RLS symptoms in hemodialysis patients. Hemodialysis patients can expect a substantial improvement of RLS symptoms after a successful kidney transplant. (C) 2002 Movement Disorder Soci

Mov. Disord.

Restless legs syndrome (RLS) is a common cause of sleep disturbance and is frequently experienced by hemodialysis patients. Factors triggering the disease in uremia have not yet been identified. To our knowledge, the course of RLS symptoms after kidney transplantation has not been investigated systematically. We investigated the clinical long-term course of RLS in hemodialysis patients who underwent kidney transplantation. Patients were given a standardized questionnaire three times: at baseline, and twice after their kidney transplants. The severity of RLS was rated by the patients (0 = no symptoms, 10 = very severe symptoms). The description of the final outcome was based on the last follow- up visit. Eleven of 64 hemodialysis patients with RLS received a transplant (5 men, 6 women; severity of RLS at baseline, 7.8 +/- 0.7 [mean +/- SEM]). In all patients, RLS symptoms disappeared within 1 to 21 days after transplantation. At follow-up visits, 4 patients whose transplanted kidneys still functioned well were still free of RLS symptoms up to the longest follow-up period of 9 years. In 3 other patients, RLS symptoms gradually reappeared (severity, 1 +/- 0). In 3 of 11 patients, the transplant failed and RLS symptoms reoccurred within10 days to 2 months (severity, 7.3 +/- 2.6). RLS symptoms reoccurred in 1 patient with failure of the transplant but disappeared again after a second, successful transplant. Kidney transplantation has a strong and positive influence on RLS symptoms in hemodialysis patients. Hemodialysis patients can expect a substantial improvement of RLS symptoms after a successful kidney transplant. (C) 2002 Movement Disorder Soci

Superconductivity for Large Scale Wind Turbines

A conceptual design has been completed for a 10MW superconducting direct drive wind turbine generator employing low temperature superconductors for the field winding. Key technology building blocks from the GE Wind and GE Healthcare businesses have been transferred across to the design of this concept machine. Wherever possible, conventional technology and production techniques have been used in order to support the case for commercialization of such a machine. Appendices A and B provide further details of the layout of the machine and the complete specification table for the concept design. Phase 1 of the program has allowed us to understand the trade-offs between the various sub-systems of such a generator and its integration with a wind turbine. A Failure Modes and Effects Analysis (FMEA) and a Technology Readiness Level (TRL) analysis have been completed resulting in the identification of high risk components within the design. The design has been analyzed from a commercial and economic point of view and Cost of Energy (COE) calculations have been carried out with the potential to reduce COE by up to 18% when compared with a permanent magnet direct drive 5MW baseline machine, resulting in a potential COE of 0.075 $/kWh. Finally, a top-level commercialization plan has been proposed to enable this technology to be transitioned to full volume production. The main body of this report will present the design processes employed and the main findings and conclusions

Primary cilia and SHH signaling impairments in human and mouse models of Parkinson's disease

Parkinson's disease (PD) as a progressive neurodegenerative disorder arises from multiple genetic and environmental factors. However, underlying pathological mechanisms remain poorly understood. Using multiplexed single-cell transcriptomics, we analyze human neural precursor cells (hNPCs) from sporadic PD (sPD) patients. Alterations in gene expression appear in pathways related to primary cilia (PC). Accordingly, in these hiPSC-derived hNPCs and neurons, we observe a shortening of PC. Additionally, we detect a shortening of PC in PINK1-deficient human cellular and mouse models of familial PD. Furthermore, in sPD models, the shortening of PC is accompanied by increased Sonic Hedgehog (SHH) signal transduction. Inhibition of this pathway rescues the alterations in PC morphology and mitochondrial dysfunction. Thus, increased SHH activity due to ciliary dysfunction may be required for the development of pathoetiological phenotypes observed in sPD like mitochondrial dysfunction. Inhibiting overactive SHH signaling may be a potential neuroprotective therapy for sPD. Here, the authors reveal using single-cell RNA sequencing that Parkinson's disease (PD) patient-derived neuronal cells show altered primary cilia morphology and signaling suggesting cilia dysfunction may underlie PD pathogenesis