49 research outputs found

    The cox1 Initiation Codon Is Created by RNA Editing in Potato Mitochondria

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    A glossary for research on human crowd dynamics

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    This article presents a glossary of terms that are frequently used in research on human crowds. This topic is inherently multidisciplinary as it includes work in and across computer science, engineering, mathematics, physics, psychology and social science, for example. We do not view the glossary presented here as a collection of finalised and formal definitions. Instead, we suggest it is a snapshot of current views and the starting point of an ongoing process that we hope will be useful in providing some guidance on the use of terminology to develop a mutual understanding across disciplines. The glossary was developed collaboratively during a multidisciplinary meeting. We deliberately allow several definitions of terms, to reflect the confluence of disciplines in the field. This also reflects the fact not all contributors necessarily agree with all definitions in this glossary

    Error distribution in randomly perturbed orbits

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    E' stata condotta una analisi di sistemi dinamici discreti 1D e 2D perturbati stocasticamente, utilizzando la fidelity per valutare la evoluzione nel tempo della distribuzione degli errori. Ci\uf2 che distingue le propriet\ue0 dinamiche delle mappe \ue8 la modalit\ue0 di rilassamento verso la distribuzione asintotica di equilibrio, che \ue8 superesponenziale per mappe caotiche, eponenziale per mappe regolari. Nelle prime esiste una soglia temporale netta, che cresce come il logaritmo dell'inverso della ampiezza del rumore, al di sotto del quale l'effetto della perturbazione \ue8 trascurabil

    Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease

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    Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulation of cholesterol and gangliosides, predominantly in endolysosomes, mitochondrial cholesterol accumulation has also been reported. As accumulation of cholesterol in mitochondria is known to impair the transport of GSH into mitochondria, resulting in mitochondrial GSH (mGSH) depletion, we investigated the impact of mGSH recovery in NPC disease. We show that GSH ethyl ester (GSH-EE), but not N-acetylcysteine (NAC), restored the mGSH pool in liver and brain of Npc1(-/-) mice and in fibroblasts from NPC patients, while both GSH-EE and NAC increased total GSH levels. GSH-EE but not NAC increased the median survival and maximal life span of Npc1(-/-) mice. Moreover, intraperitoneal therapy with GSH-EE protected against oxidative stress and oxidant-induced cell death, restored calbindin levels in cerebellar Purkinje cells and reversed locomotor impairment in Npc1(-/-) mice. High-resolution respirometry analyses revealed that GSH-EE improved oxidative phosphorylation, coupled respiration and maximal electron transfer in cerebellum of Npc1(-/-) mice. Lipidomic analyses showed that GSH-EE treatment had not effect in the profile of most sphingolipids in liver and brain, except for some particular species in brain of Npc1(-/-) mice. These findings indicate that the specific replenishment of mGSH may be a potential promising therapy for NPC disease, worth exploring alone or in combination with other options
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