Bestimmung der Citronens?ure als Pentabromaceton und ihre Anwendung auf Wein

Ohne Zusammenfassungbedeutet mit Abbildungen.     

Low frequency of p57KIP2 mutation in Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome (BWS) is an autosomal dominant disorder of increased prenatal growth and predisposition to embryonal cancers such as Wilms tumor. BWS is thought to involve one or more imprinted genes, since some patients show paternal uniparental disomy, and others show balanced germ-line chromosomal rearrangements involving the maternal chromosome. We previously mapped BWS, by genetic linkage analysis, to 11p15.5, which we and others also found to contain several imprinted genes; these include the gene for insulin-like growth factor II (IGF2) and H19, which show abnormal imprint-specific expression and/or methylation in 20% of BWS patients, and p57KIP2, a cyclin-dependent kinase inhibitor, which we found showed biallelic expression in one of nine BWS patients studied. In addition, p57KIP2 was recently reported to show mutations in two of nine BWS patients. We have now analyzed the entire coding sequence and intron-exon boundaries of p57KIP2 in 40 unrelated BWS patients. Of these patients, only two (5%) showed mutations, both involving frameshifts in the second exon. In one case, the mutation was transmitted to the proband's mother, who was also affected, from the maternal grandfather, suggesting that p57KIP2 is not imprinted in at least some affected tissues at a critical stage of development and that haploinsufficiency due to mutation of either parental allele may cause at least some features of BWS. The low frequency of p57KIP2 mutations, as well as our recent discovery of disruption of the K(v)LQT1 gene in patients with chromosomal rearrangements, suggest that BWS can involve disruption of multiple independent 11p15.5 genes.     

即插即用模块设计呼唤创新

工程师们必须适应当今以即插即用设计策略为重的市场环境，例如，大多数主力白色家电制造商越来越倾向于将电器子系统（甚至是关键子系统，如马达控制器）视为设备组件。     

Assessing students' metacognitive awareness of reading strategies.

This article describes the development and validation of a new self-report instrument, the Metacognitive Awareness of Reading Strategies Inventory, which is designed to assess adolescent and adult readers' metacognitive awareness and perceived use of reading strategies while reading academic or school-related materials. There were 3 strategy subscales or factors: Global Reading Strategies, Problem-Solving Strategies, and Support Reading Strategies. The reliability and factorial validity of the scale were demonstrated. After a brief review of the literature, the development and validation of the instrument are described, and its psychometric properties are discussed. In addition, directions for administering and scoring the instrument are provided, and suggestions for interpreting the results obtained are offered. Finally, the scales' implications for reading research and instruction are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)