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1.
We identified an expansion of the CAG trinucleotide repeat in the coding region of the Machado-Joseph disease gene in 7 of 24 American families diagnosed with autosomal dominant ataxia. All affected individuals were heterozygous for an expanded allele that ranged from 67 to more than 200 CAG repeats, whereas the normal allele had 14 to 33 repeats. In contrast to the Azorean-Portuguese origins of Machado-Joseph disease, the two largest American families were of German and Dutch-African descent. Clinical, pathologic, and genetic evaluations suggest that American families with spinocerebellar ataxia type 3 differ from those with Machado-Joseph disease by their ethnic origins, predominant spinopontine atrophy, lack of dystonic features, and larger CAG repeat expansion.  相似文献   
2.
The large and rapid variations (ramp events) of wind power output experienced in wind farms and portfolios represent one of the main challenges facing short‐term wind power forecasting. In countries with high wind power penetration, a ramp event forecasting tool is required by transmission system operators and energy traders to schedule ancillary services properly and minimize economic penalties in liberalized electricity markets, respectively. From the forecaster/modeller's point of view, locating ramp events within a wind power time series is important, because it allows them to regard meteorological processes and operational states of the wind farm in the proper time periods to analyse the ramp causes. This work introduces the ramp function as a means of characterizing the ramp performance of a wind power time series. The underlying idea is that a ramp event is characterized by high‐power output gradients evaluated under different time scales. The ramp function is based on the wavelet transform and provides a continuous index related to the ramp intensity at each time step, which permits to take into account the fuzzy limits of the ramp notion, as well as the development of new approaches to wind power ramp analysis that are not feasible from a binary classification standpoint. Several advantages of the ramp function for end‐users are outlined, and applications concerning different aspects of ramp forecasting are described for several wind farms located in Spain. Copyright © 2013 John Wiley & Sons, Ltd.  相似文献   
3.
A patient with chronic hepatitis associated with hepatitis C virus infection was observed to convert from antinuclear antibody-negative to antinuclear antibody-positive status at the time when liver cancer was detected. The newly recognized antibodies reacted with a nuclear protein doublet of 170 and 180 kDa in immunoblotting, and in fluorescence-activated flow cytometry the antigens were shown to vary in expression level in a cell cycle-related manner: minimum in G1, increasing in S, and maximum in G2 and M. In synchronized HeLa and HEp-2 cells, immunofluorescence microscopy showed uniformly distributed staining of the nucleoplasm in S-phase, with increased intensity of nucleoplasmic staining in G2, at which time nucleolar staining was also present. In M, condensed chromosomes were uniformly stained. Using previously characterized polyclonal antibodies to DNA topoisomerase II (topo II) as reference markers, the antigens recognized by the patient's serum were shown in Western blotting to have the same mobilities as DNA topo IIalpha (170 kDa) and beta (180 kDa) isoforms. The patient's serum was also highly efficient in inhibiting DNA topo II in an in vitro functional assay. Antibody to DNA topo II appeared de novo in close association with transformation to cancer, and since dysregulation of DNA topo II is considered to be involved in some forms of tumorigenesis, the observed antibody response in this patient could conceivably be an immune reaction to the abnormally regulated protein.  相似文献   
4.
Klingstedt  F.  Eränen  K.  Lindfors  L.-E.  Andersson  S.  Cider  L.  Landberg  C.  Jobson  E.  Eriksson  L.  Ilkenhans  T.  Webster  D. 《Topics in Catalysis》2004,30(1-4):27-30
Topics in Catalysis - A common-rail diesel vehicle was equipped with a full-scale Ag/alumina catalytic converter. The converter consisted of several Ag/alumina bricks, with free space in between...  相似文献   
5.
Cereal alkylresorcinols (ARs), a group of phenolic lipids mainly found in the outer parts of wheat and rye kernels, are currently being studied for possible use as biomarkers of intake of whole grain wheat and rye foods. Several different techniques have been used for quantitative AR analysis over the years, but with limited attempts to compare them. In this present study, two commonly used methods, gas chromatography and colorimetry, were evaluated and compared. Gas chromatographic and the colorimetric methods showed good agreement. The GC-method provides the total amount of AR and relative homologue composition, whereas the colorimetric method, which is based on azo-coupling of a Fast Blue salt to the hydroxyl group(s) in the alkylresorcinol molecule, only provides the total amount, but is much faster and does only requires a UV-spectrophotometer.  相似文献   
6.
In recent years some research towards developing forecasting models for wind power or energy has been carried out. In order to evaluate the prediction ability of these models, the forecasts are usually compared with those of the persistence forecast model. As shown in this article, however, it is not reasonable to use the persistence model when the forecast length is more than a few hours. Instead, a new statistical reference for predicting wind power, which basically is a weighting between the persistence and the mean of the power, is proposed. This reference forecast model is adequate for all forecast lengths and, like the persistence model, requires only measured time series as input. Copyright © 1998 John Wiley & Sons, Ltd.  相似文献   
7.
Wind power meteorology has evolved as an applied science firmly founded on boundary layer meteorology but with strong links to climatology and geography. It concerns itself with three main areas: siting of wind turbines, regional wind resource assessment and short-term prediction of the wind resource. The history, status and perspectives of wind power meteorology are presented, with emphasis on physical considerations and on its practical application. Following a global view of the wind resource, the elements of boundary layer meteorology which are most important for wind energy are reviewed: wind profiles and shear, turbulence and gust, and extreme winds. Copyright © 1998 John Wiley & Sons, Ltd.  相似文献   
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9.
Abnormal expression of the retinoblastoma protein (pRb) and cyclin D1 have been reported in a variety of malignancies, but the frequencies of these deregulations and their relation to prognosis in colorectal cancer has not been clarified. We characterised 90 colorectal cancers with respect to immunohistochemical expression of cyclin D1, pRb and Ki-67. Two of 90 (2%) tumours lacked nuclear pRb staining, indicating inactivation of the protein, while 10 (11%) expressed high levels of pRb. Abnormal expression of pRb was significantly correlated to low levels of nuclear cyclin D1 observed in 32% of the tumours. Strong nuclear cyclin D1 expression was detected in 12% of the tumours. Cytoplasmic staining of cyclin D1 was observed in 17% of the tumours, showing an inverse relationship (P = 0.006) to the Ki-67 labelling index. Eight of 11 tumours with high nuclear overexpression of cyclin D1 and both tumours with pRb defects were located in the right colon in comparison with zero of 25 in the rectum (P = 0.009). Regarding prognosis, neither pRb nor cyclin D1 expression correlated with patient survival.  相似文献   
10.
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