Suchergebnisse

By focusing primarily on the USA but also drawing upon examples from Europe and Australia, the author examines the technical, political, and constitutional issues which surround the question of high-level nuclear waste transportation. It is revealed that there are major questions of safety and public perception which are not being addressed by state institutions, and there is a discussion of the ways in which state and professional actors have attempted to limit the political discourse on these and related policy-relevant issues.

Indicates ways in which the investigation of political and economic behaviour within the world system necessarily depends on the successful manipulation of spatial data. Such data analysis-spatial analysis-involves the identification of spatial autocorrelation which can enrich understanding of conflict processes. Shows that conflict and cooperation between neighbouring states are a function of spatial structure as a whole. (PAS)

AbstractInherited disorders of mitochondrial oxidative phosphorylation are the most common group of inborn errors of metabolism and cause a wide range of clinical presentations. Mitochondrial DNA encodes 13 protein subunits required for oxidative phosphorylation plus 22 transfer RNAs and two ribosomal RNAs, and mutations in most of these genes cause human disease. Nuclear genes encode most of the protein subunits and all other proteins required for mitochondrial biogenesis and mitochondrial DNA replication and expression. Mutations in 64 nuclear genes and 34 mitochondrial genes are now known to cause mitochondrial disease and many novel mitochondrial disease genes await discovery. The genetic complexity of oxidative phosphorylation means that maternal, autosomal recessive, autosomal dominant and X-linked modes of inheritance can occur, along with de novo mutations. This complexity presents a challenge in planning efficient molecular genetic diagnosis of patients with suspected mitochondrial disease. In some situations, clinical phenotype can be strongly predictive of the underlying genotype. However, more often this is not the case and it is usually helpful, particularly with pediatric patients, to determine whether the activity of one or more of the individual oxidative phosphorylation enzymes is deficient before proceeding with mutation analysis. In this review we will summarize the genetic bases of mitochondrial disease and discuss some approaches to integrate information from clinical presentation, laboratory findings, family history, and imaging to guide molecular investigation.

This article uses methodological insights to argue for a reconceptualization of the way in which we approach spatial data, and specifically for a rejection of the primacy of the nation state as the unit of analysis. We pay particular attention to the way in which the analysis of data with a geographic basis is undertaken, particularly in situations where some form of data aggregation has been undertaken, and in cases where there exists a wide variation in the size of data units. This analysis explores the implications of this variation, employing the technique of spatial autocorrelation. Our study of peace and war in Africa extends previous analyses by Starr and Most, and illustrates the way in which the measurement of spatial autocorrelation can be employed as a powerful analytical tool to identify relations of conflict and cooperation. This article builds on these findings with some speculations on the ways in which further research on conflict and cooperation might proceed, in both technical and conceptual directions.