Explore the words cloud of the NINTCORDEV project. It provides you a very rough idea of what is the project "NINTCORDEV" about.
The following table provides information about the project.
ERASMUS UNIVERSITAIR MEDISCH CENTRUM ROTTERDAM
|Coordinator Country||Netherlands [NL]|
|Total cost||177˙598 €|
|EC max contribution||177˙598 € (100%)|
1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
|Duration (year-month-day)||from 2018-05-01 to 2020-08-21|
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|1||ERASMUS UNIVERSITAIR MEDISCH CENTRUM ROTTERDAM||NL (ROTTERDAM)||coordinator||177˙598.00|
Development of the cortex requires intricate orchestration of progenitor self-renewal and neuronal differentiation, subtype specification, migration and integration in neuronal circuits. Disturbance of any of these processes has been linked to a variety of developmental disorders, including Cornelia de Lange Syndrome (CdLS). CdLS is highlighted by intellectual disability and seizures and most frequently caused by mutations in the cohesin loading factor NIPBL. Our previous work showed that Nipbl interacts with the neuronal transcription factor Zfp609 and the Integrator complex to regulate cortical neuron migration in mouse embryos. Furthermore, INTS1 and INTS8 mutations were recently reported to cause a severe neurodevelopmental syndrome in humans.
This proposal aims to identify the human neurodevelopmental defects caused by INTS1 and INTS8 mutations and NIPBL haploinsufficiency and molecularly dissect the affected transcriptional processes. This will be accomplished by creating patient-specific INTS1, INTS8 and NIPBL mutations in human pluripotent stem cell lines, which will be differentiated into neural progenitor cells to assess effects on protein-protein interactions, long-range chromosome contacts and RNApol2 pausing and into cerebral organoids to assess the impact on different aspects of the neurodevelopmental program, including progenitor proliferation, neuronal migration and subtype specification.
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The information about "NINTCORDEV" are provided by the European Opendata Portal: CORDIS opendata.
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