18th ESGLD Workshop - September 3-6, 2011 Långvik, Finland
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ESGLD Finland 2011 will be held in Långvik, Kirkkonummi, Finland. Långvik Congress Wellness Hotel is located in the naturally beautiful environment of Kirkkonummi, at half-an-hour drive from Helsinki-Vantaa airport and from Helsinki city center. Långvik Congress Wellness Hotel offers an excellent environment for our meeting, with a combination of the facilities of a modern congress hotel and the natural beauty of the surrounding forest and the sea, by which the hotel is located. The hotel also has a Wellness-Spa and an organic restaurant where you can pamper yourself. Pictures of the hotel can be seen at. http://www.langvik.fi/en/contact/photos/
Welcome!
Local organising committee:
Anu Jalanko, Jaana Tyynelä, Aija Kyttälä, Pirkko Heikinheimo
Secretary: Tuija.Koski@thl.fi
ABSTRACT DEADLINE JUNE 15TH 2011
REGISTRATION WILL OPEN IN APRIL 2011
ESGLD Workshop, Finland 3-6.9.2011 - PRELIMINARY Programme
Saturday, September 3, 2011 |
|
4.00 pm |
Registration |
6.00 |
Get together |
8.00 |
Opening / Welcome address |
8.15 |
Opening lecture: Mart Saarma New avenues in the treatment of neurodegenerative diseases |
Sunday, September 4, 2011 |
|
08.50 – 12.20 pm |
Scientific Session I: Cell biology of lysosomes Chairperson: Volkmar Gieselmann |
08.50-09.10 am |
Katrin Marschner A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism |
09.10-09.30 |
Eeva-Liisa Eskelinen LAMP-1 and LAMP-2 in endosomal cholesterol transport |
09.30-09.50 |
Stephan Storch Lysosomal targeting and proteolytic cleavage of the lysosomal storage disease-related membrane protein CLN7 |
09.50-10.10 |
Corinne Sagne LMBD1, the protein mutated in cobalamin storage disorder (cbIF), is a highly glycosylated bona fide lysosomal membrane protein |
10.30-10.40 |
Coffee break |
10.40-11.00 |
Sandra Pohl GlcNAc-1-phosphotransferase: Role of the g-subunit for the M6P-dependent transport of lysosomal enzymes |
11.00-11.20 |
Georgia Makrypidi Isoform-dependent dephosphorylaiton of lysosomal proteins by acidic phosphatases 2 and 5 |
11.20-11.40 |
Isabell Zech Expression and intracellular transport of cerebroside sulfotransferase |
11.40-12.00 |
Carmine Settembre Genetic enhancement of autophagy as therapeutic treatment for the lysosomal storage disorders |
12.00-12.20 pm |
Alessandro Fraldi Transcriptional activation of lysosomal exocytosis promotes cellular clearance |
12.20-2.00 |
Lunch |
2.00-4.50 |
Scientific Session II: Pathophysiology of lysosomal storage diseases Chairperson: Jonathan Cooper |
2.00-2.20 |
Elise Roy GM130 dysfunction in Mucopolysaccharidosis IIIB affects lysosomogenesis |
2.20-2.40 |
Francesca Cecere MAPK dysregulation in the brain pathology of Mucopolysaccharidosis IIIB mice |
2.40-3.00 |
Alexey Pshezhetsky Protein misfolding in mucopolysaccharidosis III type C: molecular pathophysiology and therapeutic perspectives |
3.00-3.20 |
Brian W. Bigger / Alex Langford Evaluation of the behaviour of the Mucopolysaccharidosis type III A and B mouse models |
3.20-3.40
|
Björn Kowalewski Murine arylsulfatase G deficiency results in a lysosomal storage disorder |
3.40-4.10 |
Coffee break |
4.10-4.30 |
Katrin Kollmann Skeletal abnormalities in the Gnptabc.3082insC mouse model of mucolipidosis II – pathomechanisms and therapeutic strategies |
4.30-4.50 |
Emanuele Persichetti di-N-acetylchitobiase-deficient mice accumulate Man2GlcNAc2 and Man3GlcNAc2 |
04.50-6.00 |
Attended Poster Session |
6.00-6.30 |
ESGLD business meeting (for members only) |
6.00–8.00 |
Sauna |
8.00 |
Dinner |
Monday, September 5, 2011 |
|
8.30-10.00 am |
Scientific Session III: Pathophysiology of lysosomal storage diseases Chairperson Thomas Braulke |
08.30-09.00 |
Elina Ikonen Lysosomal lipid imbalance in Niemann-Pick type C disease |
09.00-09.20 |
Niklas Mattsson Amyloid metabolism in Niemann-Pick type C disease: findings in cell and animal models |
09.20-09.40 |
Maciej Lalowski Cathepsin D deficiency: system level modeling of the human disease |
09.40-10.00 |
Coffee break |
10.00-10.20 |
Kristiina Uusi-Rauva Juvenile neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endocytic compartments |
10.20–10.40 |
Andrea Dardis Characterization of the molecular mechanisms involved in the defective splicing of GAA mRNA carrying the common c.-32-13T>G mutation |
10.40-11.00 |
Paulo Gaspar Cell-type specific routing of GBA to lysosomes in LIMP-2 deficiency |
11.00–12.30 am |
Scientific Session IV: Clinical aspects of lysosomal storage diseases Chairperson Carla Hollak |
11.00-11.20 |
Catherine Caillaud Ceroid lipofuscinosis in France: from CLN1 to CLN10 |
11.20-11.40 |
S.M. Rombach The clinical course of treated and untreated patients with Fabry disease |
11.40-12.00 |
Ben PoorthuisSensitive and robust method for the quantitation of globotriaosylsphingosine in human plasma with LC/MS/MS and isotope labelled internal standard |
12.00-12.20 pm |
Derek Burke GBA 1 and 2, factors to consider in Parkinson's disease ? |
12.20–2.00 |
Lunch |
2.00–6.00 |
Social programme |
6.00-7.00 |
Attended Poster Session |
7.00-8.00 |
Sauna |
8.00 |
Gala Dinner |
Tuesday, September 6, 2011 |
|
09.00-12.40 am |
Scientific Session V: Therapy of lysosomal storage diseases Chairperson Paola Di Natale |
09.00-09.20 am |
Seng H. Cheng CNS expression of glucocerebrosidase corrects a-synuclein pathology and memory deficits in a mouse model of Gaucher-related synucleinopathy |
09.20-09.40 |
Nicolina Sorrentino A novel systemic AAV-mediated gene therapy to cross the blood brain barrier and rescue the brain pathology in a lysosomal storage disorder |
09.40-10.00 |
Margherita Neri / Alessandra Ricca Neural stem cell gene therapy ameliorates pathology and function in a mouse model of Globoid Cell Leukodystrophy |
10.00-10.20 |
Jonathan Cooper Moving towards therapy for infantile neuronal ceroid lipofuscinosis |
10.20-10.40 |
Coffee break |
10.40-11.00 |
Ulrich Matzner Therapeutic efficacy of enzyme replacement therapy in an aggravated mouse model of MLD declines with age |
11.00-11.20 |
Annika Böckenhoff Enzyme replacement therapy of metachromatic leukodystrophy: ASA fusion proteins for improved delivery of enzyme activity |
11.20-11.40 |
G.E. Lindthorst Antibody status causes a sustained lack of urinary Bg3 clearance and reduced IysoGb3 reduction despite long term ERT for fabry disease in male patients |
11.40-12.00 |
MJ Ferraz Inhibition of glucosylceramide synthase leads to storage reduction in Fabry mice |
12.00-12.20 pm |
Eduard Paschke Fluorous iminoalditols are effective pharmacological chaperones in fibroblasts from patients with juvenile or adult GM1-Gangliosidosis |
12.20-12.40 |
Heidi Repo Systematic study on chaperone lead compounds for acid a-glucosidase |
12.40-1.00 |
Young Scientist's award |
1.00-2.00 |
Lunch and departure |
