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

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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