Zobrazení zdroje stránky Stefansendejesus2676

Many genodermatoses exhibit abnormal teeth findings. Studies examining these entities are scarce and narrow in their scope. This paper reviews the evolution, development, and structure of the tooth and provides a summary of genodermatoses with aberrant dental findings. The latter are classified according to the abnormal dental findings periodontal disease, anodontia/oligodontia/hypodontia, polydontia, enamel hypoplasia, natal teeth, dental pits, and others. Finally, we provide an algorithm that dermatologists and dentists can follow to better recognize genodermatoses with dental involvement. © 2020 John Wiley &amp; Sons A/S. Published by John Wiley &amp; Sons Ltd. All rights reserved.BACKGROUND Clinical features of neurofibromatosis type 1 (NF1) are diverse and include plexiform neurofibromas (PNs). Increasing knowledge of the molecular pathways involved in the growth of NF1 related tumours and the advent of molecularly targeted anti-cancer drugs have resulted in the development of potential medical treatments to cease PNs progression in these patients. The inactivation of genes responsible for phakomatoses have been linked in a common biochemical pathway to a deregulation of the mammalian target of rapamycin (mTOR) signalling. Cell lines derived from NF1-related tumours have shown to be sensitive to the mTOR inhibitor rapamycin. CASE PRESENTATION We describe an 11 years old girl diagnosed of NF1 with a symptomatic progressive PN involving the left brachial plexus treated with oral everolimus, a mTOR inhibitor, at an initial dose of 3 mg/m2 per day. [https://www.selleckchem.com/products/pd173212.html see more] A magnetic resonance imaging performed three months after introduction of everolimus, revealed reduction of the tumour volume with further shrinkage over the next 18 months while on treatment. She achieved pain alleviation and recovered tactile sensation. Everolimus was well tolerated. CONCLUSION mTOR inhibitors may represent a treatment option to promote regression of PNs associated to NF1. This article is protected by copyright. All rights reserved.While the use of visible light in conjunction with transition metal catalysis offers powerful opportunities to switch between on / off states of catalytic activity, the next frontier would be the ability to switch the actual function of the catalyst and resulting products. Here we report such an example of multi-dimensional catalysis. Featuring an easily prepared, bench-stable cobalt(I) hydride complex in conjunction with pinacolborane, we can switch the reaction outcome between two widely employed transformations, olefin migration and hydroboration, with visible light as the trigger. © 2020 WILEY-VCH Verlag GmbH &amp; Co. KGaA, Weinheim.Human Schwann cells (hSCs) can be isolated directly from peripheral nerve and cultured using methods similar to those used for SCs from other species. Yet, important interspecies differences are revealed when the primary or expanded hSCs are compared to their nonhuman counterparts. This review addresses the special properties of nerve-derived hSCs that have resulted to date from both in vitro studies and in vivo research on cell transplantation in animal models and human subjects. A consensus has yet to emerge about the essential attributes of cultured normal hSCs. Thus, an emphasis is placed on the importance of validating hSC cultures by means of purity, identity, and biological activity to reliably use them as in vitro models of the SC phenotype and cell therapy products for injury repair. Combining traditional immunological methods, high-resolution omics approaches, and assorted cell-based assays is so far the best approach to unequivocally identify hSC populations obtained by direct isolation or derivation from stem cells. Special considerations are required to understand and manage the variability and heterogeneity proper of donor batches, as well as to evaluate risk factors. This is particularly important if the intended use of the hSCs is implantation in the human body, diagnosis of disease, or drug testing aimed at targeting any aspect of SC function in human patients. To conclude, in view of their unique properties, new concepts and methods are needed to better understand the biology of hSCs and exploit their full potential in basic science and regenerative medicine. © 2020 Wiley Periodicals, Inc.Psychological theories posit that the hippocampus rapidly forms associations among ongoing events as they unfold. During a subsequent maturation process, so-called systems memory consolidation, these associations are gradually stabilized within distributed neocortical circuits through close interactions between the hippocampus and neocortex. In the past 50&nbsp;years, a major effort in neurobiological research has been directed towards translating these descriptive accounts into tangible, biological processes in the brain. Until the early 2000s, most studies exclusively focused on examining whether the hippocampus becomes unnecessary for memory retrieval once the memory has been consolidated. With recent methodological advances, however, the field shifted attention to several other theoretical accounts and began to uncover the genetic, physiological and structural underpinnings of systems memory consolidation at an unprecedented level of precision. Here I review these neurobiological findings in the past 15&nbsp;years within a framework of six essential predictions extracted from the psychological theories. Genetic approaches have made it possible to tag neurons that were activated during memory encoding and investigate their physiological and genetic profiles as well as reactivation patterns during subsequent retrieval. In parallel, electrophysiological and imaging approaches detected signs of the gradual refinement of memory representations and its underlying hippocampal-neocortical dialogue in millisecond-resolved neural firing patterns, the inter-region coupling of neural activity, across-day stability of neural ensemble activity and functional connectome. This summary represents substantial progress in our understanding of neurobiological mechanisms of systems memory consolidation whilst also identifying several essential remaining questions for future investigations. © 2020 Federation of European Neuroscience Societies and John Wiley &amp; Sons Ltd.