MGS AND DISEASE

Analysis of the pathological implications of the abnormal accumulation of glycogen in specific cell types

 Coordinatore FUNDACIO INSTITUT DE RECERCA BIOMEDICA (IRB BARCELONA) 

 Organization address address: CARRER BALDIRI REIXAC 10-12 PARC SCIENTIFIC DE BARCELONA
city: BARCELONA
postcode: 8028

contact info
Titolo: Mr.
Nome: Alexandre
Cognome: Puerto Valdivieso
Email: send email
Telefono: 34934039810
Fax: 34934037140

 Nazionalità Coordinatore Spain [ES]
 Totale costo 212˙554 €
 EC contributo 212˙554 €
 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-2009-IEF
 Funding Scheme MC-IEF
 Anno di inizio 2010
 Periodo (anno-mese-giorno) 2010-07-01   -   2012-07-13

 Partecipanti

# participant  country  role  EC contrib. [€] 
1    FUNDACIO INSTITUT DE RECERCA BIOMEDICA (IRB BARCELONA)

 Organization address address: CARRER BALDIRI REIXAC 10-12 PARC SCIENTIFIC DE BARCELONA
city: BARCELONA
postcode: 8028

contact info
Titolo: Mr.
Nome: Alexandre
Cognome: Puerto Valdivieso
Email: send email
Telefono: 34934039810
Fax: 34934037140

ES (BARCELONA) coordinator 212˙554.00

Mappa


 Word cloud

Esplora la "nuvola delle parole (Word Cloud) per avere un'idea di massima del progetto.

human    tissues    mainly    scientific    accumulation    models    mechanisms    mouse    apoptosis    cell    manager    mgs    group    liver    applicant    disease    glycogen    expressed    muscle   

 Obiettivo del progetto (Objective)

'Glucose is stored in specialized tissues (mainly liver and muscle) in the form of a branched polymer called glycogen. Glycogen Synthase is the only enzyme able to catalyze this polymerization and its two isoforms (LGS and MGS) are mainly expressed in liver and in muscle, respectively. However, MGS is also expressed in all other tissues, regardless of their limited capacity to produce Glycogen. MGS activity is tightly controlled by several mechanisms. Recent results from the receiving group show that demise of these mechanisms in neurons causes apoptosis and may be the cause of Lafora disease. Our working hypothesis is that deregulation of MGS protein levels and/or activity is causative of several pathologies. Therefore, we aim to dissect the consequences of the loss of regulation of MGS in several cell types and tissues, using a state-of-the-art genetic approach. Our results will bring new evidence for the understanding of many unresolved human health conditions, and help to identify potential therapeutical strategies. The specific research objectives are: 1. To elucidate the mechanism by which the accumulation of glycogen triggers apoptosis. 2. To study whether aberrant accumulation of glycogen is responsible for some degenerative conditions. 3. To establish whether GS has a moonlighting function. These questions will be addressed both in vitro, using cell culture and diverse cell biology techniques, and in vivo, using newly available conditional mouse models. The applicant will join a group of reference in the study of the metabolism of glycogen, and will offer his extensive experience in the generation and use of mouse models for human disease. It is the applicant’s aim to become a skilled, independent research manager, either as a group leader or as scientific manager for research institutions. This project will provide the applicant with excellent scientific and managerial training opportunities.'

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