Guillain-Barre syndrome (GBS)

A 57-year-old Caucasian gentleman presented with bilateral progressive distal upper limb paraesthesiae, which he described as a feeling of “heaviness” followed by distal lower limb and mild tongue parasthaesia. He complained of dysaesthetic symptoms in his upper limbs with intermittent burning and tingling and autonomic disturbances such as excessive sweating of the face, hands and legs. He later developed epigastric pain that radiated to the chest, which was not related to exercise. According to the patient, symptoms got worse after taking the influenza vaccine. On examination, he had gait disturbance with weaker left lower limb muscles. During his stay in hospital, he also developed slight dysarthria and diplopia, together with urinary retention and constipation. He had had a similar, though much less severe, episode six years previously where he was diagnosed with Guillain-Barré Syndrome based on his clinical features, EMG result and his high protein levels in the CSF (more than 8g/L). He was treated with IVIG and recovered completely. This was his second presentation of neuromuscular weakness and he was referred for immunoglobulin treatment and intensive physiotherapy. GBS is an acute inflammatory immune-mediated disorder affecting the peripheral nervous system. GBS typically manifests as sudden distal symmetrical parasthaesia that ascends to the upper limbs and progresses to weakness. A few patients undergo sensory dysfunction especially in the demyelinating forms of GBS. About a third of hospitalised patients are mechanically ventilated due to diaphragmatic, respiratory and oropharyngeal muscle weaknesspeer-reviewe

Zelluläre Antwort auf mitochondrialen Stress infolge einer Nonsense-Mutation in der Cytochrom-c-Oxidase Untereinheit 1 (COX1) in humanen Cybridzellen

Punktmutationen in der mitochondrialen DNA (mtDNA) führen zur Bildung defekter Proteine und folglich zu Atmungskettendefekten, welche verschiedenste Erkrankungen im Menschen verursachen. Bisher wurde angenommen, dass mutierte Proteine nicht in die Atmungskettenkomplexe eingebaut werden können und durch mitochondriale Qualitäts- kontrollproteasen abgebaut werden. Um die Mechanismen des selektiven Proteinabbaus und der mitochondrialen Stressantwort detaillierter zu verstehen, wurde eine Cybridzell- linie mit einer G6930A Punktmutation in der Cytochrom-c-Oxidase Untereinheit 1 des Komplex IV untersucht. Durch die Mutation entsteht ein Stopp-Codon und folglich ein um 30 % verkürztes Protein, welches jedoch in nicht nachweisbarer Menge vorliegt. In G6930A Cybridzellen liegen neben dem Komplex IV-Defekt auch verminderte Aktivitä- ten und Mengen der Atmungskettenkomplexe I, II und III vor. Um die nukleäre Antwort auf den mitochondrialen Stress zu analysieren, wurde eine Genexpressionsanalyse (mRNA-Array) durchgeführt und eine reduzierte Genexpression vieler nukleär kodierter Atmungskettenuntereinheiten festgestellt. Eine kompensatori- sche Hochregulation der mitochondrialen Biogenese konnte somit ausgeschlossen wer- den. Zusätzlich waren die mRNA- und Proteinmengen der mitochondrialen Qualitäts- kontrollproteasen AFG3L2 und YME1L1 hochreguliert. Um die Rolle und den Effekt der Protease AFG3L2 im Abbau von Atmungskettenuntereinheiten zu untersuchen, wur- den Pulse Chase Experimente nach transienter Transfektion von AFG3L2-Varianten und transientem AFG3L2-Knockdown durchgeführt. Während die Überexpression der Wild- typform von AFG3L2 in einem erhöhten Abbau von Atmungskettenuntereinheiten re- sultierte, führte sowohl die Überexpression der dominant negativen AFG3L2-Variante (AFG3L2E408Q ) als auch der Knockdown zu einer gesteigerten Stabilität. Die äußerst wichtige Rolle von AFG3L2 im Abbau von Atmungskettenuntereinheiten konnte auf die- se Weise demonstriert werden. Darüber hinaus wurde das verkürzte COX1 Protein, sowie andere Atmungskettenuntereinheiten der Komplexe I, IV und V mit Hilfe von Pulldown- Experimenten als Substrate von AFG3L2 identifiziert. Die in dieser Arbeit erhaltenen Ergebnisse lassen auf einen gegenseitigen Austausch von Informationen zwischen Mitochondrien und Nukleus schließen, um die Anreicherung von fehlgefalteten und nicht-assemblierten Atmungskettenuntereinheiten und einen da- mit verbundenen negativen Effekt in den Mitochondrien zu verhindern. Dieses Ziel wird zum einen durch die herunterregulierte Genexpression von kernkodierten mitochondrialen Atmungskettenuntereinheiten und zum anderen durch die Hochregulation der mitochon- drialen Qualitätskontrollproteasen erreicht

Non degeneracy of critical points of the Robin function with respect to deformations of the domain

We show a result of genericity for non degenerate critical points of the Robin function with respect to deformations of the domai

“SOCIAL AGRICULTURE”: A PATTERN BETWEEN FARM INNOVATION, SOCIAL RESPONSIBILITY AND MULTIFUNCTIONALITY

Rural context and agricultural process are assuming growing credibility as a mean to promote well being and social inclusion in the population as a whole. Fattoria solidale del Circeo” constitutes one of the many examples of social agriculture that have risen in the recent years in Italy. Differently from the general case, it is a large farm which is moving from a conventional an intensive farming system to another model, without abandoning its entrepreneurial nature. Even if it is not easy to assign a theoretic paradigm, it could be useful both in order to understand it and to give correct instruments to policy makers. So we can try to put the Fattoria Solidale del Circeo’s experience in a theoretic context. It seems to us that at least three different models can be applied: the well-established Schumpeterian model of innovation; the Corporate Social Responsibility, the Multifunctionality of agriculture.Social agriculture, Innovation, Corporate Social Responsibility, Multifunctionality of Agriculture, Community/Rural/Urban Development, Institutional and Behavioral Economics, Labor and Human Capital,

Foreign Investments in Philippine Agriculture

This article measures the amount of foreign participation, direct and indirecin Philippine agriculture. However, due to insufficient time and inadequate data, its aim has been reduced to aid further research on the effects of foreign participation in Philippine agriculture.agriculture sector, investment

Foreign Investments in Philippine Agriculture

This article measures the amount of foreign participation, direct and indirecin Philippine agriculture. However, due to insufficient time and inadequate data, its aim has been reduced to aid further research on the effects of foreign participation in Philippine agriculture.agriculture sector, investment

Blow-up phenomena for linearly perturbed Yamabe problem on manifolds with umbilic boundary

We build blowing-up solutions for linear perturbation of the Yamabe problem on manifolds with umbilic boundary, provided the Weyl tensor is nonzero everywhere on the boundary and the dimension of the manifold is n>10

The role of the scalar curvature in some singularly perturbed coupled elliptic systems on Riemannian manifolds

Given a 3-dimensional Riemannian manifold (M,g), we investigate the existence of positive solutions of singularly perturbed Klein-Gordon-Maxwell systems and Schroedinger-Maxwell systems on M, with a subcritical nonlinearity. We prove that when the perturbation parameter epsilon is small enough, any stable critical point x_0 of the scalar curvature of the manifold (M,g) generates a positive solution (u_eps,v_eps) to both the systems such that u_eps concentrates at xi_0 as epsilon goes to zero