Molecular forms of porphobilinogen deaminase in acute intermittent porphyria. A study by Western immunoblotting

Acute intermittent porphyria is an inborn error of haem synthesis which is transmitted as a dominant character with variable phenotypic expression. The disorder is caused by a partial deficiency of porphobilinogen deaminase in all tissues so far studied. The nature of the enzymatic deficiency of porphobilinogen deaminase in haemolysates from patients with acute intermittent porphyria was examined by the use of monospecific antibody probes. In affected heterozygotes from three British pedigrees of diverse ancestry, the catalytic deficiency of porphobilinogen deaminase was accompanied by diminished enzyme protein, as determined by radial immunodiffusion. No evidence of functionally attenuated enzyme was demonstrable by kinetic studies. The molecular forms of the residual enzyme were investigated in red cell extracts and in lysed preparations of reticulocytes by a sensitive Western blotting procedure. This revealed the presence of reduced amounts of porphobilinogen deaminase polypeptide co-migrating with wild type enzyme (Mr approximately 40,000), and no evidence of variant forms in situ. The studies show that porphobilinogen deaminase deficiency in acute intermittent porphyria is commonly associated with a CRM-phenotype. The residual activity under these circumstances is thus related to expression of a single normal allele, since sensitive techniques detected neither aberrant nor degraded forms of the enzyme in erythroid tissues.

Essays on labour market dualisation in Western Europe: active labour market policies, temporary work regulation and inequality

European labour markets are increasingly divided between insiders in full-time permanent employment and outsiders in precarious work or unemployment. Using quantitative as well as qualitative methods, this thesis investigates the determinants and consequences of labour market policies that target these outsiders in three separate papers. The first paper looks at Active Labour Market Policies (ALMPs) that target the unemployed. It shows that left and right-wing parties choose different types of ALMPs depending on the policy and the welfare regime in which the party is located. These findings reconcile the conflicting theoretical expectations from the Power Resource approach and the insider-outsider theory. The second paper considers the regulation and protection of the temporary work sector. It solves the puzzle of temporary re-regulation in France, which contrasts with most other European countries that have deregulated temporary work. Permanent workers are adversely affected by the expansion of temporary work in France because of general skills and low wage coordination. The interests of temporary and permanent workers for re-regulation therefore overlap in France and left governments have an incentive to re-regulate the sector. The third paper then investigates what determines inequality between median and bottom income workers. It shows that non-inclusive economic coordination increases inequality in the absence of compensating institutions such as minimum wage regulation. The deregulation of temporary work as well as spending on employment incentives and rehabilitation also has adverse effects on inequality. Thus, policies that target outsiders have important economic effects on the rest of the workforce. Three broader contributions can be identified. First, welfare state policies may not always be in the interests of labour, so left parties may not always promote them. Second, the interests of insiders and outsiders are not necessarily at odds. Third, economic coordination may not be conducive to egalitarianism where it is not inclusive.

Simulated changes in the relationship between tropical ocean temperatures and the western African monsoon during the mid-Holocene

Results from nine coupled ocean-atmosphere simulations have been used to investigate changes in the relationship between the variability of monsoon precipitation over western Africa and tropical sea surface temperatures (SSTs) between the mid-Holocene and the present day. Although the influence of tropical SSTs on the African monsoon is generally overestimated in the control simulations, the models reproduce aspects of the observed modes of variability. Thus, most models reproduce the observed negative correlation between western Sahelian precipitation and SST anomalies in the eastern tropical Pacific, and many of them capture the positive correlation between SST anomalies in the eastern tropical Atlantic and precipitation over the Guinea coastal region. Although the response of individual model to the change in orbital forcing between 6 ka and present differs somewhat, eight of the models show that the strength of the teleconnection between SSTs in the eastern tropical Pacific and Sahelian precipitation is weaker in the mid-Holocene. Some of the models imply that this weakening was associated with a shift towards longer time periods (from 3–5 years in the control simulations toward 4–10 years in the mid-Holocene simulations). The simulated reduction in the teleconnection between eastern tropical Pacific SSTs and Sahelian precipitation appears to be primarily related to a reduction in the atmospheric circulation bridge between the Pacific and West Africa but, depending on the model, other mechanisms such as increased importance of other modes of tropical ocean variability or increased local recycling of monsoonal precipitation can also play a role.

Response to comment on “The response of vegetation on the Andean flank in western Amazonia to Pleistocene climate change” – M.L. Cárdenas et al., Science 331, 1055 (25 February 2011)”.

Puyasena et al. question our interpretation of climate-driven vegetation change on the Andean flank in western Amazonia during the middle Pleistocene and suggest that the use of Podocarpus spp. as a proxy of past climate change should be reassessed. We defend our assertion that vegetation change at the Erazo study site was predominantly driven by climate change due to concomitant changes recorded by multiple taxa in the fossil record.

The response of vegetation on the Andean flank in western Amazonia to Pleistocene climate change

A reconstruction of past environmental change from Ecuador reveals the response of lower montane forest on the Andean flank in western Amazonia to glacial-interglacial global climate change. Radiometric dating of volcanic ash indicates that deposition occurred ~324,000 to 193,000 years ago during parts of Marine Isotope Stages 9, 7, and 6. Fossil pollen and wood preserved within organic sediments suggest that the composition of the forest altered radically in response to glacial-interglacial climate change. The presence of Podocarpus macrofossils ~1000 meters below the lower limit of their modern distribution indicates a relative cooling of at least 5°C during glacials and persistence of wet conditions. Interglacial deposits contain thermophilic palms suggesting warm and wet climates. Hence, global temperature change can radically alter vegetation communities and biodiversity in this region.

Changing HRM systems in two Russian oil companies: Western hegemony or Russian spetsifika?

We contrast attempts to introduce what were seen as sophisticated Western-style human resource management (HRM) systems into two Russian oil companies – a joint venture with a Western multinational corporation (TNK-BP) and a wholly Russian-owned company (Yukos). The drivers for Western hegemony within the joint venture, heavily influenced by expatriates and the established HRM processes introduced by the Western parent, were counteracted to a significant degree by the Russian spetsifika – the peculiarly Russian way of thinking and doing things. In contrast, developments were absorbed faster in the more authoritarian Russian-owned company. The research adds to the theoretical debate about international knowledge transfer and provides detailed empirical data to support our understanding of the effect of both organizational and cultural context on the knowledge-transfer mechanisms of local and multinational companies. As the analysis is based on the perspective of senior local nationals, we also address a relatively under-researched area in the international HRM literature which mostly relies on empirical data collected from expatriates and those based solely in multinational headquarters.

Predictability of the western North Pacific summer climate associated with different ENSO phases by ENSEMBLES multi-model seasonal forecasts

Predictability of the western North Pacific (WNP) summer climate associated with different El Niño–Southern Oscillation (ENSO) phases is investigated in this study based on the 1-month lead retrospective forecasts of five state-of-the-art coupled models from ENSEMBLES. During the period from 1960 to 2005, the models well capture the WNP summer climate anomalies during most of years in different ENSO phases except the La Niña decaying summers. In the El Niño developing, El Niño decaying and La Niña developing summers, the prediction skills are high for the WNP summer monsoon index (WNPMI), with the prediction correlation larger than 0.7. The high prediction skills of the lower-tropospheric circulation during these phases are found mainly over the tropical western Pacific Ocean, South China Sea and subtropical WNP. These good predictions correspond well to their close teleconnection with ENSO and the high prediction skills of tropical SSTs. By contrast, for the La Niña decaying summers, the prediction skills are considerably low with the prediction correlation for the WNPMI near to zero and low prediction skills around the Philippines and subtropical WNP. These poor predictions relate to the weak summer anomalies of the WNPMI during the La Niña decaying years and no significant connections between the WNP lower-tropospheric circulation anomalies and the SSTs over the tropical central and eastern Pacific Ocean in observations. However, the models tend to predict an apparent anomalous cyclone over the WNP during the La Niña decaying years, indicating a linearity of the circulation response over WNP in the models prediction in comparison with that during the El Niño decaying years which differs from observations. In addition, the models show considerable capability in describing the WNP summer anomalies during the ENSO neutral summers. These anomalies are related to the positive feedback between the WNP lower-tropospheric circulation and the local SSTs. The models can capture this positive feedback but with some uncertainties from different ensemble members during the ENSO neutral summers.

The 2013 hot, dry summer in Western Europe

The European summer of 2013 was marked by hot and dry conditions in Western Europe associated with a northward shifted Atlantic storm track and a positive phase of the SNAO. Model results suggest that, relative to a 1964–93 reference period, changes in SST/SIE explain 63% (±26%) of the area-averaged warming signal over Western Europe, with the remaining 37% (±29%) explained by the direct impact of changes in anthropogenic radiative forcings from GHG and aerosols. The results further suggest that the anomalous atmospheric circulation, and associated low rainfall, were also influenced both by changes in SST/SIE and by the direct impact of changes in radiative forcings; however, the magnitude of the forced signals in these variables is much less, relative to internal variability, than for surface air temperature. Further evidence suggests that changes in North Atlantic SST were likely an important factor in explaining the striking contrast between the European summers of 2013 and that of 2012. A major area for further work is to understand more completely the mechanisms that explain these influences.

The emergence of Agribusiness in Europe and the development of the Western European broiler chicken industry, 1945-73

This article presents new data on the emergence and growth of the leading Western European poultry industries after 1945. It shows that those countries where poultry output grew most quickly – especially the UK, Italy and Spain – were also the countries where the agricultural sectors adopted US technologies and US agribusiness organizational structures most vigorously. Elsewhere in Western Europe, poultry output grew much less quickly and the adoption of agribusiness structures lagged behind. By contrast, the poultry sector in the USSR was based on the Soviet collectivist system. This was the largest poultry sector in Europe, but also much less efficient. The article suggests therefore that the diffusion of agribusiness and the increase in poultry output were deeply entwined across Europe, with potentially important consequences for the different roles and impacts of agribusiness in Europe.

The rise of Western rationalism: Paul Feyerabend’s story

I summarise certain aspects of Paul Feyerabend’s account of the development of Western rationalism, show the ways in which that account is supposed to run up against an alternative, that of Karl Popper, and then try to give a preliminary comparison of the two. My interest is primarily in whether what Feyerabend called his ‘story’ constitutes a possible history of our epistemic concepts and their trajectory. I express some grave reservations about that story, and about Feyerabend’s framework, finding Popper’s views less problematic here. However, I also suggest that one important aspect of Feyerabend’s material, his treatment of religious belief, can be given an interpretation which makes it tenable, and perhaps preferable to a Popperian approach.

Western Britain in late antiquity

The relevance of the concept of ‘Late Antiquity’ to fifth- and sixth-century Western Britain is demonstrated with reference to the archaeology of the British kingdom of Dumnonia, and then used to reinterpret portable material culture. Themes discussed include the dating of Palestinian amphorae in Britain, the extent of the settlement at Tintagel, tin as a motivation for Byzantine trade, the re-use of Roman-period artefacts, and ‘Anglo-Saxon’ artefacts on Western British sites. The central paradoxes of Late Antiquity: simultaneous conservatism and fluidity, continuity and innovation, are seen to illuminate ‘Dark Age’ Britain and offer new avenues for future research.

Large-scale dynamics associated with clustering of extratropical cyclones affecting Western Europe

Some recent winters in Western Europe have been characterized by the occurrence of multiple extratropical cyclones following a similar path. The occurrence of such cyclone clusters leads to large socio-economic impacts due to damaging winds, storm surges, and floods. Recent studies have statistically characterized the clustering of extratropical cyclones over the North Atlantic and Europe and hypothesized potential physical mechanisms responsible for their formation. Here we analyze 4 months characterized by multiple cyclones over Western Europe (February 1990, January 1993, December 1999, and January 2007). The evolution of the eddy driven jet stream, Rossby wave-breaking, and upstream/downstream cyclone development are investigated to infer the role of the large-scale flow and to determine if clustered cyclones are related to each other. Results suggest that optimal conditions for the occurrence of cyclone clusters are provided by a recurrent extension of an intensified eddy driven jet toward Western Europe lasting at least 1 week. Multiple Rossby wave-breaking occurrences on both the poleward and equatorward flanks of the jet contribute to the development of these anomalous large-scale conditions. The analysis of the daily weather charts reveals that upstream cyclone development (secondary cyclogenesis, where new cyclones are generated on the trailing fronts of mature cyclones) is strongly related to cyclone clustering, with multiple cyclones developing on a single jet streak. The present analysis permits a deeper understanding of the physical reasons leading to the occurrence of cyclone families over the North Atlantic, enabling a better estimation of the associated cumulative risk over Europe.