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BaskAtax

Modulation of Basket cell microcircuits in cerebellar cortex paroxysmal ataxia disorders

Total Cost €

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EC-Contrib. €

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Partnership

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 BaskAtax project word cloud

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molecule    kv1    model    combination    vivo    cutting    therapies    disorder    reveals    terminals    prevalent    inhibitory    action    ataxias    human    ataxia    rare    regulation    basket    behavioural    network    unforeseen    cell    electrophysiology    msc    biochemistry    episodic    share    microscopy    possibly    specialized    recurring    global    neuropharmacology    similarities    paroxysmal    background    mouse    pinceau    local    techniques    disorders    train    attacks    neurological    dominantly    edge    ea1    dysfunction    paroxysms    experiments    subunit    unknown    candidate    whereby    channel    animal    altered    extensive    building    microcircuits    mutations    potassium    vitro    unclear    linked    function    synaptic    small    firing    prototypic    cortex    conventional    photon    drawing    deficits    epilepsy    understand    models    mechanism    harbouring    inherited    assay    cerebellar    therapeutic    mutation    purkinje    hereditary    missense    incoordination    me    migraine    structure    extend    map    group   

Project "BaskAtax" data sheet

The following table provides information about the project.

Coordinator
UNIVERSITY COLLEGE LONDON 

Organization address
address: GOWER STREET
city: LONDON
postcode: WC1E 6BT
website: n.a.

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 United Kingdom [UK]
 Project website https://www.ucl.ac.uk/ion/research/synaptopathies/principal-investigators/dimitri-m-kullmann
 Total cost 195˙454 €
 EC max contribution 195˙454 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2016
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2017
 Duration (year-month-day) from 2017-10-01   to  2019-09-30

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    UNIVERSITY COLLEGE LONDON UK (LONDON) coordinator 195˙454.00

Map

 Project objective

The episodic ataxias are a group of hereditary conditions with recurring paroxysms of cerebellar dysfunction. They are rare; however they share important similarities to other more prevalent paroxysmal disorders such as migraine and epilepsy. The prototypic cerebellar cortex paroxysmal disorder is Episodic ataxia 1 (EA1), which is linked to dominantly inherited missense mutations in the Kv1.1 potassium channel subunit. Work from an animal model harbouring a human EA1 mutation reveals altered synaptic function at basket cell terminals in the cerebellar cortex. However how EA1 mutations affect basket cell regulation of Purkinje cell firing is unclear, possibly due to unforeseen changes in a specialized inhibitory structure called the pinceau. Furthermore the mechanism whereby local synaptic deficits extend to global cerebellar cortex network dysfunction during attacks of incoordination is unknown. This MSC research action aims to understand these processes using a combination of cutting edge in vitro and in vivo techniques. Using advanced electrophysiology techniques, I will assay basket cell pinceau function in mouse models of EA1, then using multi-photon and conventional microscopy I will map local basket cell microcircuits. Finally building on in vitro experiments I will assay candidate small molecule therapies in vivo, both with electrophysiology and with behavioural test of cerebellar coordination. The project allows me to train in state-of-the-art in vitro and in vivo methods while drawing on my extensive background in neuropharmacology, biochemistry electrophysiology. This research action will also advance our understanding of paroxysmal neurological disorders and identify new therapeutic targets.

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

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