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Summary Sphingolipidoses are caused by recessively inherited deficiencies of lysosomal hydrolases. The clinical backgrounds of and current biochemical and genetic approaches to the different forms and variants of gangliosidoses, trihexosylceramidosis (Fabry's disease), galactosylceramidosis (Krabbe's disease), sulfatidoses (metachromatic leukodystrophies), glucosylceramidosis (Gaucher's disease), sphingomyelinoses (Niemann-Pick disease) and ceramidosis (Farber's disease) are presented.Based on a report given at the Fifteenth Meeting of the German Society of Anthropology and Human Genetics in Hamburg, 1977Professor Pilz died tragically on March 27, 1978This study was supported by the Deutsche Forschungsgemeinschaft (Sonderforschungsbereich 33 Nervensystem und biologische Information)  相似文献   

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DNA microarray technologies have evolved rapidly to become a key high-throughput technology for the simultaneous measurement of the relative expression levels of thousands of individual genes. However, despite the widespread adoption of DNA microarray technology, there remains considerable uncertainty and scepticism regarding data obtained using these technologies. Comparing results from seemingly identical experiments from different laboratories or even from different days can prove challenging; these challenges increase further when data from different array platforms need to be compared. To comply with emerging regulations, the quality of the data generated from array experiments needs to be clearly demonstrated. This review describes several initiatives that aim to improve confidence in data generated by array experiments, including initiatives to develop standards for data reporting and storage, external spike-in controls, quality control procedures, best practice guidelines, and quality metrics.  相似文献   

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Cardiopulmonary bypass (CPB) procedures require a blood-gas exchanger (oxygenator) to temporarily replace the respiratory function of the lungs. In the past the majority of CPB procedures have been carried out with bubble oxygenators which effect gas exchange by dispersion of bubbles into the blood. Membrane oxygenators, on the other hand, utilize a hydrophobic gas permeable membrane between the blood and gas phases.Bubble oxygenators are being superseded by membrane types for CPB due to improvements in membrane technology and mass transfer efficiency. These advances are reviewed in this paper and are illustrated by reference to the gas exchange and operating characteristics of a number of clinical oxygenators designed for adult CPB.Membrane oxygenatorsare also being used for long-term support in the treatment of acute respiratory failure. Operated in a partial bypass circuit, the oxygenator may have to function for several days or weeks. In one particular treatment method, the rate of spontaneous breathing is controlled by the partial or total removal of the metabolic CO2 production by the membrane oxygenator. For this method, known as extracorporeal CO2 removal (ECCO2R), the oxygenator must be optimized for CO2 transfer at low blood flow rates. The suitability of clinical oxygenators for ECCO2R is discussed in terms of gas exchange and functionality over a prolonged operation.  相似文献   

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The cryopreservation of oocytes of most animal species remains a challenge due to their complex structure. Equilibrium freezing is not satisfactory because oocytes seem to be damaged by exposure for several minutes to temperatures near 0 degrees C. Therefore, cryopreservation of oocytes by vitrification--especially the use of very high cooling rates of oocytes suspended in extremely small volumes of various cryoprotective additive modifications--seems the most appropriate method.  相似文献   

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1 动物学实验教学存在的问题和实行改革的必要 传统的动物学实验一般以形态解剖和动物分类为主,课程教学内容多为演示性和验证性实验.这些实验对培养学生的基本实验技能、基本实验方法、常用仪器的使用以及数据处理是十分必要的,但模式比较单一.教学中综合类、研究类实验几乎是空白,未能反映现代动物学新发展内容,这对培养学生科研和创新能力不利(乔淑芬,2007).因此,改造传统的实验教学内容和实验技术方法,建立新型的适应学生能力培养、鼓励探索的多元化实验教学模式、培养学生的创新能力是非常必要的.3  相似文献   

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《Journal of Zoology》1999,248(3):419-419
The following Applications were published on 31 March 1999 in Vol. 56, Part 1 of the Bulletin of Zoological Nomenclature . Comment or advice on any of these applications is invited for publication in the Bulletin and should be sent to the Executive Secretary (I.C.Z.N.), c/o The Natural History Museum, Cromwell Road, London SW7 5BD, U.K. (e-mail: iczn@nhm.ac.uk ).  相似文献   

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