Search

Field measurements of long-term dynamic phenomena inside an industrial power plant connected to the public distribution network were carried out in order to assess the performance of the power system with its internal generating units. To this end, perturbations were intentionally produced by suitable manipulation of the control variables such as boiler master control (fuel), feedwater and speed governor. The long-term dynamic is of strong interest since, even if the supply in the industrial plant survives short term transients (transient stability), then it could be unable to maintain its frequency due to the inadequacy of the boiler power response. The recorded variables were: electric active power delivered by the two generating units; frequency; steam and feedwater pressure; steam temperature; steam, feedwater and fuel flow; and boiler level. Such measurement results could later be used to validate a model of a typical industrial power plant boiler suitable for the simulation of all kinds of long-term phenomena. (5 pages)

Noonan, LEOPARD, and cardiofaciocutaneous Syndromes (NS, LS, and CFCS) are developmental disorders with overlapping features including distinctive facial dysmorphia, reduced growth, cardiac defects, skeletal and ectodermal anomalies, and variable cognitive deficits. Dysregulated RAS-mitogen-activated protein kinase (MAPK) signal traffic has been established to represent the molecular pathogenic cause underlying these conditions. To investigate the phenotypic spectrum and molecular diversity of germline mutations affecting BRAF, which encodes a serine/threonine kinase functioning as a RAS effector frequently mutated in CFCS, subjects with a diagnosis of NS (N = 270), LS (N = 6), and CFCS (N = 33), and no mutation in PTPN11, SOS1, KRAS, RAF1, MEK1, or MEK2, were screened for the entire coding sequence of the gene. Besides the expected high prevalence of mutations observed among CFCS patients (5296), a de novo heterozygous missense change was identified in one subject with LS (17%) and five individuals with NS (1.9%). Mutations mapped to multiple protein domains and largely did not overlap with cancer-associated defects. NS-causing mutations had not been documented in CFCS, suggesting that the phenotypes arising from germline BRAF defects might be allele specific. Selected mutant BRAF proteins promoted variable gain of function of the kinase, but appeared less activating compared to the recurrent cancer-associated p.Val600Glu mutant. Our findings provide evidence for a wide phenotypic diversity associated with mutations affecting BRAF, and occurrence of a clinical continuum associated with these molecular lesions. Hum Mutat 30, 695-702, 2009. (C) 2009 Wiley-Liss, Inc.