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MiniBRAIN SIGNED

Investigating the pathogenic mechanisms underlying TUBB2B-related brain malformations using induced pluripotent stem cells and cerebral organoids.

Total Cost €

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EC-Contrib. €

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Partnership

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 MiniBRAIN project word cloud

Explore the words cloud of the MiniBRAIN project. It provides you a very rough idea of what is the project "MiniBRAIN" about.

mts    preliminary    me    cell    cas9    patients    co    community    hypothesis    dr    lineage    stemming    global    keays    elucidate    generate    organising    structural    division    mutations    mechanisms    genome    certain    impaired    pluripotent    phenotypes    embryonic    abnormal    pathogenic    crispr    functional    organoid    differentiated    fibroblasts    cells    genotypes    tubb2b    brains    suggests    disease    imba    cos    scientific    stem    neurogenesis    gene    neuronal    investigation    neural    reputable    pioneers    dynamics    expressed    mutant    tubulin    cortical    david    editing    microtubules    self    leaders    cortex    lab    microscopy    technologies    employ    techniques    malformations    hosted    normal    roles    perform    imp    disorders    immunohistochemistry    close    ipscs    wild    mechanism    knoblich    mutation    vienna    controls    highlights    mt    brain    proliferation    data    human    figure    recapitulate    communication    aggregated    mini    examine    cerebral    organisation    cycle    migration    subsequently    severe    encodes    isogenic    underlying    line   

Project "MiniBRAIN" data sheet

The following table provides information about the project.

Coordinator
FORSCHUNGSINSTITUT FUR MOLEKULARE PATHOLOGIE GESELLSCHAFT MBH 

Organization address
address: CAMPUS-VIENNA-BIOCENTER 1
city: WIEN
postcode: 1030
website: www.imp.ac.at

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country Austria [AT]
 Total cost 166˙156 €
 EC max contribution 166˙156 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2017
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2019
 Duration (year-month-day) from 2019-03-01   to  2021-02-28

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    FORSCHUNGSINSTITUT FUR MOLEKULARE PATHOLOGIE GESELLSCHAFT MBH AT (WIEN) coordinator 166˙156.00

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 Project objective

Mutations in TUBB2B are associated with a range of malformations of cortical development: severe structural brain disorders stemming from abnormal cerebral cortex formation. Functional investigation of TUBB2B mutations will enable us to elucidate distinct pathogenic mechanisms underlying various malformations and advance our understanding of normal brain development. TUBB2B is highly expressed during embryonic brain development. It encodes a major component of microtubules (MTs), which perform essential roles during neuronal proliferation, neuronal migration and cortical organisation. I have obtained preliminary data in non-neuronal cells that suggests certain (but not all) TUBB2B-related malformations result from impaired cell division during neurogenesis. This highlights a potential disease-specific mechanism. I will investigate this hypothesis using state-of-the-art induced pluripotent stem cells (iPSCs) and cerebral organoid (COs) technologies, more relevant to brain development. I will generate iPSCs from fibroblasts obtained from patients with specific TUBB2B genotypes and brain phenotypes. I will use CRISPR/Cas9 genome editing to generate isogenic controls (in addition to a generic wild type line). Mutant and control iPSCs will be differentiated into a neural lineage to study effects of mutations on cell cycle and MT dynamics. Subsequently, differentiated cells will be aggregated into COs; self-organising ‘mini-brains’ that recapitulate human brain development and disease. I will employ immunohistochemistry and microscopy to examine TUBB2B mutation effects on neuronal proliferation, migration and organisation. I will hosted by Dr David Keays (IMP, Vienna). His lab are global leaders in tubulin-gene research and work in close collaboration with the pioneers in CO techniques (Knoblich Lab, IMBA, Vienna). Dissemination and communication of results will impact the scientific community, promote EU-based research and establish me as a reputable figure in the field.

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The information about "MINIBRAIN" are provided by the European Opendata Portal: CORDIS opendata.

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