3Dpaleo.net, una plataforma web de fósiles en 3D hiperrealista para fomentar la difusión y el conocimiento del patrimonio paleontológico

3Dpaleo.net es una plataforma web en fase de desarrollo que muestra modelos tridimensionales hiperrealistas y en alta definición de algunos de fósiles más emblemáticos del Museo de Ciencias Naturales de la Universidad de Zaragoza y del Museo del Jurásico de Asturias (MUJA), colaboradores de la primera fase del proyecto. Se trata de una iniciativa promovida por la empresa Paleoymás con el fin de explorar y explotar las posibilidades de las nuevas tecnologías, aplicándolas a la divulgación de la paleontología y a la mejora de técnicas expositivas y de difusión del conocimiento. 3Dpaleo.net facilita, a través de internet, la visualización de los fósiles y de algunos de sus detalles más interesantes. 3Dpaleo.net is a website, under development, that shows high definition hyperrealistic 3D models of some emblematic fossils of the museums that have collaborated in this first stage of the project: The Natural Sciences Museum of the University of Zaragoza and the Jurassic Museum of Asturias (MUJA). It is promoted by Paleoymás with the aim of exploring and developing the possibilities of new technologies, applying them to the popularization of paleontology and the improvement of both; expositive technics and knowledge sharing. 3Dpaleo.net helps through the internet to display fossils and some of their most interesting details

Mitochondrial cristae-remodeling protein OPA1 in POMC neurons couples Ca2+ homeostasis with adipose tissue lipolysis

© 2021 The Authors.Appropriate cristae remodeling is a determinant of mitochondrial function and bioenergetics and thus represents a crucial process for cellular metabolic adaptations. Here, we show that mitochondrial cristae architecture and expression of the master cristae-remodeling protein OPA1 in proopiomelanocortin (POMC) neurons, which are key metabolic sensors implicated in energy balance control, is affected by fluctuations in nutrient availability. Genetic inactivation of OPA1 in POMC neurons causes dramatic alterations in cristae topology, mitochondrial Ca2+ handling, reduction in alpha-melanocyte stimulating hormone (α-MSH) in target areas, hyperphagia, and attenuated white adipose tissue (WAT) lipolysis resulting in obesity. Pharmacological blockade of mitochondrial Ca2+ influx restores α-MSH and the lipolytic program, while improving the metabolic defects of mutant mice. Chemogenetic manipulation of POMC neurons confirms a role in lipolysis control. Our results unveil a novel axis that connects OPA1 in POMC neurons with mitochondrial cristae, Ca2+ homeostasis, and WAT lipolysis in the regulation of energy balance.This work was supported by Agencia Estatal de Investigación y Fondo Social Europeo, Proyecto BFU2016-76973-R FEDER (C.V.A.); AG052005, AG052986, AG051459, DK111178 from NIH and NKFI-KKP-126998 from Hungarian National Research, Development and Innovation Office (T.L.H.); MR/P009824/2 from Medical Research Council UK (G.D.); and Ayudas Fundación BBVA a Investigadores y Creadores Culturales (2015), European Research Council (ERC) under the European Union’s Horizon 2020 Research And Innovation Program (grant agreement 725004) and CERCA Programme/Generalitat de Catalunya (M.C.). A.O. is supported by a Miguel Servet contract (CP19/00083) from Instituto de Salud Carlos III and co-financed by FEDER

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

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

ASMase regulates autophagy and lysosomal membrane permeabilization and its inhibition prevents early stage non-alcoholic steatohepatitis

Abstract not availableRaquel Fucho, Laura Martínez, Anna Baulies, Sandra Torres, Nuria Tarrats, Anna Fernandez, Vicente Ribas, Alma M. Astudillo, Jesús Balsinde, Pablo Garcia-Rovés, Montserrat Elena, Ina Bergheim, Sophie Lotersztajn, Christian Trautwein, Hanna Appelqvist, Adrienne W. Paton, James C. Paton, Mark J. Czaja, Neil Kaplowitz, Jose C. Fernandez-Checa, Carmen García-Rui