A ricardian analysis of the impact of climate change on permanent crops in a mediterranean region

This is the first study which explores the impact of climate change in Sicily, a small Mediterranean region of Southern Europe. According to research, Mediterranean area has shown large climate shifts in the last century and it has been identified as one of the most prominent “Hot-Spots” in future climate change projections. Since agriculture is an economic activity which strongly depends on climate setting and is particularly responsive to climate changes, it is important to understand how such changes may affect agricultural profitability in the Mediterranean region. The aim of the present study is to assess the expected impact of climate change on permanent crops cultivated in Sicilian region (Southern Italy). By using data from Farm Accountancy Data Network and Ensembles climatic projections for 2021-2050 period, we showed that the impact of climate change is prominent in this region. However, crops respond to climatic variations in a different manner, highlighting that unlike the strong reduction in profitability of grapevine and citrus tree, the predicted average Net Revenue of olive tree is almost the same as in the reference period (1961-1990)

Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I

Glutaric acidemia type I (GA-I) is an inherited disorder of lysine and tryptophan metabolism presenting with striatal lesions anatomically and symptomatically similar to Huntington disease. Affected children commonly suffer acute brain injury in the context of a catabolic state associated with nonspecific illness. The mechanisms underlying injury and age-dependent susceptibility have been unknown, and lack of a diagnostic marker heralding brain injury has impeded intervention efforts. Using a mouse model of GA-I, we show that pathologic events began in the neuronal compartment while enhanced lysine accumulation in the immature brain allowed increased glutaric acid production resulting in age-dependent injury. Glutamate and GABA depletion correlated with brain glutaric acid accumulation and could be monitored in vivo by proton nuclear magnetic resonance (1H NMR) spectroscopy as a diagnostic marker. Blocking brain lysine uptake reduced glutaric acid levels and brain injury. These findings provide what we believe are new monitoring and treatment strategies that may translate for use in human GA-I

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with lifethreatening cerebral oedema and dysmyelination in affected individuals. Treatment requires life-long dietary restriction and monitoring of branched-chain amino acids to avoid brain injury. Despite careful management, children commonly suffer metabolic decompensation in the context of catabolic stress associated with non-specific illness. The mechanisms underlying this decompensation and brain injury are poorly understood. Using recently developed mouse models of classic and intermediate maple syrup urine disease, we assessed biochemical, behavioural and neuropathological changes that occurred during encephalopathy in these mice. Here, we show that rapid brain leucine accumulation displaces other essential amino acids resulting in neurotransmitter depletion and disruption of normal brain growth and development. A novel approach of administering norleucine to heterozygous mothers of classic maple syrup urine disease pups reduced branched-chain amino acid accumulation in milk as well as blood and brain of these pups to enhance survival. Similarly, norleucine substantially delayed encephalopathy in intermediate maple syrup urine disease mice placed on a high protein diet that mimics the catabolic stress shown to cause encephalopathy in human maple syrup urine disease. Current findings suggest two converging mechanisms of brain injury in maple syrup urine disease including: (i) neurotransmitter deficiencies and growth restriction associated with branchedchain amino acid accumulation and (ii) energy deprivation through Krebs cycle disruption associated with branched-chain ketoacid accumulation. Both classic and intermediate models appear to be useful to study the mechanism of brain injury and potential treatment strategies for maple syrup urine disease. Norleucine should be further tested as a potential treatment to prevent encephalopathy in children with maple syrup urine disease during catabolic stress

Neonatal Astrocyte Damage Is Sufficient to Trigger Progressive Striatal Degeneration in a Rat Model of Glutaric Acidemia-I

BACKGROUND: We have investigated whether an acute metabolic damage to astrocytes during the neonatal period may critically disrupt subsequent brain development, leading to neurodevelopmental disorders. Astrocytes are vulnerable to glutaric acid (GA), a dicarboxylic acid that accumulates in millimolar concentrations in Glutaric Acidemia I (GA-I), an inherited neurometabolic childhood disease characterized by degeneration of striatal neurons. While GA induces astrocyte mitochondrial dysfunction, oxidative stress and subsequent increased proliferation, it is presently unknown whether such astrocytic dysfunction is sufficient to trigger striatal neuronal loss. METHODOLOGY/PRINCIPAL FINDINGS: A single intracerebroventricular dose of GA was administered to rat pups at postnatal day 0 (P0) to induce an acute, transient rise of GA levels in the central nervous system (CNS). GA administration potently elicited proliferation of astrocytes expressing S100β followed by GFAP astrocytosis and nitrotyrosine staining lasting until P45. Remarkably, GA did not induce acute neuronal loss assessed by FluoroJade C and NeuN cell count. Instead, neuronal death appeared several days after GA treatment and progressively increased until P45, suggesting a delayed onset of striatal degeneration. The axonal bundles perforating the striatum were disorganized following GA administration. In cell cultures, GA did not affect survival of either striatal astrocytes or neurons, even at high concentrations. However, astrocytes activated by a short exposure to GA caused neuronal death through the production of soluble factors. Iron porphyrin antioxidants prevented GA-induced astrocyte proliferation and striatal degeneration in vivo, as well as astrocyte-mediated neuronal loss in vitro. CONCLUSIONS/SIGNIFICANCE: Taken together, these results indicate that a transient metabolic insult with GA induces long lasting phenotypic changes in astrocytes that cause them to promote striatal neuronal death. Pharmacological protection of astrocytes with antioxidants during encephalopatic crisis may prevent astrocyte dysfunction and the ineluctable progression of disease in children with GA-I

Diagnosis and management of glutaric aciduria type I – revised recommendations

Glutaric aciduria type I (synonym, glutaric acidemia type I) is a rare organic aciduria. Untreated patients characteristically develop dystonia during infancy resulting in a high morbidity and mortality. The neuropathological correlate is striatal injury which results from encephalopathic crises precipitated by infectious diseases, immunizations and surgery during a finite period of brain development, or develops insidiously without clinically apparent crises. Glutaric aciduria type I is caused by inherited deficiency of glutaryl-CoA dehydrogenase which is involved in the catabolic pathways of L-lysine, L-hydroxylysine and L-tryptophan. This defect gives rise to elevated glutaric acid, 3-hydroxyglutaric acid, glutaconic acid, and glutarylcarnitine which can be detected by gas chromatography/mass spectrometry (organic acids) or tandem mass spectrometry (acylcarnitines). Glutaric aciduria type I is included in the panel of diseases that are identified by expanded newborn screening in some countries. It has been shown that in the majority of neonatally diagnosed patients striatal injury can be prevented by combined metabolic treatment. Metabolic treatment that includes a low lysine diet, carnitine supplementation and intensified emergency treatment during acute episodes of intercurrent illness should be introduced and monitored by an experienced interdisciplinary team. However, initiation of treatment after the onset of symptoms is generally not effective in preventing permanent damage. Secondary dystonia is often difficult to treat, and the efficacy of available drugs cannot be predicted precisely in individual patients. The major aim of this revision is to re-evaluate the previous diagnostic and therapeutic recommendations for patients with this disease and incorporate new research findings into the guideline

Impact of COVID-19 on farming systems in Europe through the lens of resilience thinking

CONTEXT Resilience is the ability to deal with shocks and stresses, including the unknown and previously unimaginable, such as the Covid-19 crisis. OBJECTIVE This paper assesses (i) how different farming systems were exposed to the crisis, (ii) which resilience capacities were revealed and (iii) how resilience was enabled or constrained by the farming systems’ social and institutional environment. METHODS The 11 farming systems included have been analysed since 2017. This allows a comparison of pre-Covid-19 findings and the Covid-19 crisis. Pre-Covid findings are from the SURE-Farm systematic sustainability and resilience assessment. For Covid-19 a special data collection was carried out during the early stage of lockdowns. RESULTS AND CONCLUSIONS Our case studies found limited impact of Covid-19 on the production and delivery of food and other agricultural products. This was due to either little exposure or the agile activation of robustness capacities of the farming systems in combination with an enabling institutional environment. Revealed capacities were mainly based on already existing connectedness among farmers and more broadly in value chains. Across cases, the experience of the crisis triggered reflexivity about the operation of the farming systems. Recurring topics were the need for shorter chains, more fairness towards farmers, and less dependence on migrant workers. However, actors in the farming systems and the enabling environment generally focused on the immediate issues and gave little real consideration to long-term implications and challenges. Hence, adaptive or transformative capacities were much less on display than coping capacities. The comparison with pre-Covid findings mostly showed similarities. If challenges, such as shortage of labour, already played before the crisis, they persisted during the crisis. Also, the eminent role of resilience attributes was confirmed. In cases with high connectedness and diversity we found that these system characteristics importantly contributed to dealing with the crisis. Also the focus on coping capacities was already visible before the crisis. We are not sure yet whether the focus on short-term robustness just reflects the higher visibility and urgency of shocks compared to slow processes that undermine or threaten important system functions, or whether they betray an imbalance in resilience capacities at the expense of adaptability and transformability. SIGNIFICANCE Our analysis indicates that if transformations are required, e.g. to respond to concerns about transnational value chains and future pandemics from zoonosis, the transformative capacity of many farming systems needs to be actively enhanced through an enabling environment

The sicilian cooperative system of wine production: the strategic choices and performance analyses of a case study

Purpose: The purpose of this paper is to verify whether, besides the traditional organisational models mainly implemented by wine-making cooperatives, more modern and hybrid organisational forms can be profitably applied within an increasingly competitive wine market. Design/methodology/approach: The study outlined in this paper deployed a mixed method. Specifically, an archived analysis, a survey and a descriptive case study (including visits, interviews and documentary analysis) were the methodological techniques used in this study, which were “in series but integrated” between themselves. In this paper, the landscape of Sicilian wine cooperatives is described by collating and processing different types of statistical sources, which have been integrated by direct surveys undertaken in 2017. Thereafter, the study focussed on a wine cooperative with a specific business model and a strategic edge by analysing its strategic choices and main structural and governance characteristics. Within this case study, a financial ratio analysis, which was based on 2011-2017 financial statements, was conducted to analyse the profitability, financial balance, capital structure and debt relationships of the wine cooperative. Findings: The Sicilian wine cooperative system is still predominantly characterised by partial and vertical integration, implemented by cooperatives which elect to sell mainly bulk wine to wine merchants. In such a context, there is scope for other degrees of integration and strategic inter-firm alliances; the latter includes “vertical quasi-integration”. The study demonstrated how the wine cooperative under investigation is overcoming the structural problems of the regional wine sector and why it is retaining such a strategic alliance with one of the most important Italian wine conglomerates. Indeed, it has acquired greater strength and reliability since its collaboration with the aforementioned wine company. Thus, total revenue and the company’s market share of packaged wine have increased. However, there are still margins for improving sales’ profitability. Research limitations/implications: This study has territorial limitations but Sicilian wine cooperatives generally play an important role in the regional, Italian and European wine industries. As such, this research should be considered as an exploratory study, deserving further investigation into different strategic choices within the wine cooperative system by performing cross-case comparisons. Results may also be useful in orienting cooperative strategies in Sicily (or further afield) to small-to-medium wine cooperatives, often lacking specific abilities relating to the distribution, marketing and selling of their wine. Public agricultural policies may also be enlightened by these research pathways. Originality/value: The authors contend that their study provides hitherto missing information relating to inter-firm strategic alliances, which wine cooperatives might implement to enhance their competitiveness and survive in the long-run

EU income stabilization tool: potential impacts, financial sustainability and farmer\u2019s risk aversion

The Income Stabilization Tool, a risk management scheme introduced within the Common Agricultural Policy (CAP) 2014\u20132020, could help European Union farmers manage the income risks they face. This study assesses the potential impact of implementing this tool through the maximum level of contribution to the fund which determines an indifference to participate in the fund and its financial sustainability. The study relies on an expected utility approach and assesses the variability of loss ratios over time using a sample of Italian hazelnut farms as a case study. The participation depends on the level of farmers' contributions and their degree of risk aversion. However, the CAP public support makes the scheme financially sustainable