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

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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