{"id":23191,"date":"2023-09-12T08:45:43","date_gmt":"2023-09-12T06:45:43","guid":{"rendered":"https:\/\/idibell.cat\/es\/?post_type=agenda&#038;p=23191"},"modified":"2024-01-18T11:34:20","modified_gmt":"2024-01-18T10:34:20","slug":"idibellseminars-ipsc-and-genome-editing-as-tools-to-generate-human-models-of-neuronopathic-lysosomal-storage-disorders","status":"publish","type":"agenda","link":"https:\/\/idibell.cat\/es\/agenda\/idibellseminars-ipsc-and-genome-editing-as-tools-to-generate-human-models-of-neuronopathic-lysosomal-storage-disorders\/","title":{"rendered":"#IDIBELLseminars: iPSC and genome editing as tools to generate human models of neuronopathic lysosomal storage disorders"},"content":{"rendered":"<p>To better understand the pathophysiological mechanisms of rare human neurological disorders, we need to develop better human in vitro models. For that purpose, we combine iPSCs and genome editing with a variety of protocols to differentiate these cells towards human brain cells to better understand neuronopathic lysosomal storage disorders, which can affect children early in life. For many years, these disorders have been considered as neurodegenerative and caused by neuronal dysfunction, however, recent evidence supports the idea that these disorders can affect brain developmental processes and that different brain cell types can contribute and promote neuronal dysfunction. We have generated several iPSC lines from patients of Gaucher Disease, the most common lysosomal storage disorder, and differentiated them towards neuronal and glial cells to investigate the role of each cell type in the disease. Importantly, from the same iPSC lines we have generated brain organoids and identified an unknown impairment in the development of the interneuron lineage.<\/p>\n<p><em>Hosted by Mireya Plass &#8211; Gene Regulation of Cell Identity group<\/em><\/p>\n","protected":false},"featured_media":23192,"template":"","meta":{"site-sidebar-layout":"default","site-content-layout":"","ast-site-content-layout":"default","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"default","adv-header-id-meta":"","stick-header-meta":"default","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"set","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}}},"tags":[382],"class_list":["post-23191","agenda","type-agenda","status-publish","has-post-thumbnail","hentry","tag-regenerativemedicine"],"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"_links":{"self":[{"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/agenda\/23191"}],"collection":[{"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/agenda"}],"about":[{"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/types\/agenda"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/media\/23192"}],"wp:attachment":[{"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/media?parent=23191"}],"wp:term":[{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/idibell.cat\/es\/wp-json\/wp\/v2\/tags?post=23191"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}