Transformative investment: New rules for investing in sustainability transitions

Closing the financial gap for promoting systemic socio-economic transformations to achieve sustainability requires both a substantial increase in investment levels and a qualitative change in investment strategies. In this Perspective, we elaborate on this claim and discuss why existing investment approaches that aim to make positive contributions to sustainability are unlikely to foster the systemic transformations needed for sustainability. Qualitative change means changing the current rules that guide investment practices and we outline a new set of rules that should guide transformative investment. These rules are based on the well-established socio-technical sustainability transition theory and the recent development of a theory of deep transitions. We explain why these transformative investment rules offer a promising alternative base for assessing investment opportunities and monitoring progress toward the multi-system changes required to achieve a socially just deep transition to sustainability

User-made immobilities: a transitions perspective

In this paper we aim to conceptualize the role of users in creating, expanding and stabilizing the automobility system. Drawing on transition studies we offer a typology of user roles including user-producers, user-legitimators, user-intermediaries, user-citizens and user-consumers, and explore it on the historical transition to the automobile regime in the USA. We find that users play an important role during the entire transition process, but some roles are more salient than others in particular phases. Another finding is that the success of the transition depends on the stabilization of the emerging regime that will trigger upscaling in terms of the numbers of adopters. The findings are used to reflect on potential crossovers between transitions and mobilities research

Microcephaly with simplified gyration, epilepsy, and infantile diabetes linked to inappropriate apoptosis of neural progenitors

We describe a syndrome of primary microcephaly with simplified gyral pattern in combination with severe infantile epileptic encephalopathy and early-onset permanent diabetes in two unrelated consanguineous families with at least three affected children. Linkage analysis revealed a region on chromosome 18 with a significant LOD score of 4.3. In this area, two homozygous nonconserved missense mutations in immediate early response 3 interacting protein 1 (IER3IP1) were found in patients from both families. IER3IP1 is highly expressed in the fetal brain cortex and fetal pancreas and is thought to be involved in endoplasmic reticulum stress response. We reported one of these families previously in a paper on Wolcott-Rallison syndrome (WRS). WRS is characterized by increased apoptotic cell death as part of an uncontrolled unfolded protein response. Increased apoptosis has been shown to be a cause of microcephaly in animal models. An autopsy specimen from one patient showed increased apoptosis in the cerebral cortex and pancreas beta cells, implicating premature cell death as the pathogenetic mechanism. Both patient fibroblasts and control fibroblasts treated with siRNA specific for IER3IP1 showed an increased susceptibility to apoptotic cell death under stress conditions in comparison to controls. This directly implicates IER3IP1 in the regulation of cell survival. Identification of IER3IP1 mutations sheds light on the mechanisms of brain development and on the pathogenesis of infantile epilepsy and early-onset permanent diabetes.Facultad de Ciencias Médica

Microcephaly with simplified gyration, epilepsy, and infantile diabetes linked to inappropriate apoptosis of neural progenitors

We describe a syndrome of primary microcephaly with simplified gyral pattern in combination with severe infantile epileptic encephalopathy and early-onset permanent diabetes in two unrelated consanguineous families with at least three affected children. Linkage analysis revealed a region on chromosome 18 with a significant LOD score of 4.3. In this area, two homozygous nonconserved missense mutations in immediate early response 3 interacting protein 1 (IER3IP1) were found in patients from both families. IER3IP1 is highly expressed in the fetal brain cortex and fetal pancreas and is thought to be involved in endoplasmic reticulum stress response. We reported one of these families previously in a paper on Wolcott-Rallison syndrome (WRS). WRS is characterized by increased apoptotic cell death as part of an uncontrolled unfolded protein response. Increased apoptosis has been shown to be a cause of microcephaly in animal models. An autopsy specimen from one patient showed increased apoptosis in the cerebral cortex and pancreas beta cells, implicating premature cell death as the pathogenetic mechanism. Both patient fibroblasts and control fibroblasts treated with siRNA specific for IER3IP1 showed an increased susceptibility to apoptotic cell death under stress conditions in comparison to controls. This directly implicates IER3IP1 in the regulation of cell survival. Identification of IER3IP1 mutations sheds light on the mechanisms of brain development and on the pathogenesis of infantile epilepsy and early-onset permanent diabetes.Facultad de Ciencias Médica

Sit-to-Stand Analysis in the Wild using Silhouettes for Longitudinal Health Monitoring

We present the first fully automated Sit-to-Stand or Stand-to-Sit (StS) analysis framework for long-term monitoring of patients in free-living environments using video silhouettes. Our method adopts a coarse-to-fine time localisation approach, where a deep learning classifier identifies possible StS sequences from silhouettes, and a smart peak detection stage provides fine localisation based on 3D bounding boxes. We tested our method on data from real homes of participants and monitored patients undergoing total hip or knee replacement. Our results show 94.4% overall accuracy in the coarse localisation and an error of 0.026 m/s in the speed of ascent measurement, highlighting important trends in the recuperation of patients who underwent surgery

Transformative policy mixes in socio-technical scenarios: the case of the low-carbon transition of the German electricity system (2010-2050)

Much research and policy advice for addressing climate change has focused on developing model-based scenarios to identify pathways towards achieving decarbonisation targets. The paper's first aim is to complement such model-based analysis with insights from socio-technical transition analysis to develop socio-technical storylines that show how low-carbon transitions can be implemented. Our second aim is to explore how policymakers could govern such transition processes through transformative policy mixes. We take the example of the transition of the German electricity system towards renewable energies, and elaborate two transition pathways which are assumed to achieve an 80% reduction in greenhouse gas emissions by 2050, but differ in terms of lead actors, depth and scope of change: the first pathway captures the substitution of technological components (pathway A), while the second aims at broader system transformation (pathway B). We find that multi-dimensional socio-technical change (pathway B) requires greater emphasis on societal experimentation and a more proactive role for anticipatory deliberation processes from the outset. In contrast, shifting gear from a new entrant friendly past trajectory to an incumbent dominated pathway (pathway A) requires agency from incumbents and is associated with regime stabilizing instruments defending the old regime while simultaneously fulfilling decarbonisation as additional success criteria