ELANSCI

Role of Lgi and Adam proteins in nerve development and function

 Coordinatore ERASMUS UNIVERSITAIR MEDISCH CENTRUM ROTTERDAM 

 Organization address address: 's Gravendijkwal 230
city: ROTTERDAM
postcode: 3015CE

contact info
Titolo: Ms.
Nome: Nuran
Cognome: Tuzen
Email: send email
Telefono: -7043190
Fax: -7044722

 Nazionalità Coordinatore Netherlands [NL]
 Totale costo 161˙162 €
 EC contributo 161˙162 €
 Programma FP7-PEOPLE
Specific programme "People" implementing the Seventh Framework Programme of the European Community for research, technological development and demonstration activities (2007 to 2013)
 Code Call FP7-PEOPLE-IEF-2008
 Funding Scheme MC-IEF
 Anno di inizio 2009
 Periodo (anno-mese-giorno) 2009-07-01   -   2011-10-31

 Partecipanti

# participant  country  role  EC contrib. [€] 
1    ERASMUS UNIVERSITAIR MEDISCH CENTRUM ROTTERDAM

 Organization address address: 's Gravendijkwal 230
city: ROTTERDAM
postcode: 3015CE

contact info
Titolo: Ms.
Nome: Nuran
Cognome: Tuzen
Email: send email
Telefono: -7043190
Fax: -7044722

NL (ROTTERDAM) coordinator 161˙162.47

Mappa


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function    demonstrated    mutant    interaction    peripheral    metalloprotease    cells    suggesting    adam    sc    members    nervous    pns    expressed    lgi    mice    proteins    sorting    axonal    cellular    neurons    nerve    molecular    family    mechanisms   

 Obiettivo del progetto (Objective)

'Schwann cells (SC) are the most important accessory cells to neurons in the peripheral nervous system (PNS) and are involved in every aspect of nerve structure, function and regeneration. Dr Meijer’s group has identified a novel SC expressed factor, Lgi4, involved in nerve development and function. The Lgi4 gene is mutated in a natural mouse mutant called Claw Paw, which exhibits delayed axonal sorting and hypomyelination in the PNS. Lgi4 is a member of a small family of secreted proteins mainly expressed in the nervous system. While mutations in Lgi1 have been implicated in epilepsy in humans, virtually nothing is known about the other two members of the family; Lgi2 and Lgi3. These genes are expressed during development in primary sensory neurons, suggesting that they, together with Lgi4, are involved in diverse aspects of development and function of the PNS. Some or all of these functions could be mediated through interaction with members of the Adam (A Disintergin And Metalloprotease) family of transmembrane proteins. It has been demonstrated that Lgi1 binds to the metalloprotease inactive member Adam22. Recent results from the host laboratory have demonstrated that Lgi4, 2 and 3 also bind Adam22. Adam22 mutant mice have a phenotype that resembles that of Lgi4 mutant mice, suggesting a possible interaction during nerve development. The cellular processes and molecular mechanisms that are influenced by this interaction are at present unclear. Therefore, the major aim of this research proposal is to unravel the cellular and molecular mechanisms through which Lgi4-Adam22 interaction contributes to proper axonal sorting and myelin formation. Insight into the role of Lgi proteins in peripheral nerve development will be instrumental in developing novel therapeutic strategies to aid nerve repair and function and, in addition, will inform us about the role these proteins play in eleptogenesis.'

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