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

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

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 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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