Explore the words cloud of the LYSOBONE project. It provides you a very rough idea of what is the project "LYSOBONE" about.
The following table provides information about the project.
Coordinator |
UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF
Organization address contact info |
Coordinator Country | Germany [DE] |
Total cost | 159˙460 € |
EC max contribution | 159˙460 € (100%) |
Programme |
1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility) |
Code Call | H2020-MSCA-IF-2017 |
Funding Scheme | MSCA-IF-EF-ST |
Starting year | 2018 |
Duration (year-month-day) | from 2018-03-01 to 2020-02-29 |
Take a look of project's partnership.
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1 | UNIVERSITAETSKLINIKUM HAMBURG-EPPENDORF | DE (HAMBURG) | coordinator | 159˙460.00 |
Mucopolysaccharidosis-VI (MPS-VI) is a lysosomal storage disorder predominantly affecting skeletal remodelling and caused by pathogenic mutations in the ARSB gene, encoding the glycosaminoglycan-degrading enzyme arylsulfatase B. Preliminary analysis of Arsb-deficient mice unraveled a skeletal phenotype of similar severity and with lysosomal storage defects in different skeletal cell types. This Arsb-deficient mouse model therefore provides an excellent tool to understand the skeletal and non-skeletal pathologies related to MPS-VI and to address the clinically relevant question, if enzyme replacement therapy (ERT) can influence the existing (non-)skeletal pathologies in lysosomal storage disorders. Moreover, another major objective is to unravel the cellular consequences of Arsb-deficiency on a molecular level and to investigate mechanisms of ARSB uptake and lysosomal delivery in different primary cell types of Arsb-deficient mice. This project will therefore certainly generate knowledge with relevance for other (skeletal) disorders as well, as the cellular defects in MPS-VI are closely linked to that of two of the most prevalent disorders in the European Union, i.e. osteoporosis and osteoarthritis. Next to its scientific goals, this proposal aims to offer a broad personal training program for me as a postdoctoral researcher as well. Handling this project will not only offer an intensive scientific training (intellectual-methodological), but also improve my skills regarding project management (administrative-financial) and writing. As a two-way transfer, my previously acquired knowledge on the genetics of skeletal disorders and related experimental skills will be transferred to the host organization. A final goal of this proposal is to disseminate the acquired data towards specialized scientific audiences (scientific conferences and publications) and propagate these data towards non-expert audiences (patient meetings, press releases) as well.
year | authors and title | journal | last update |
---|---|---|---|
2020 |
Gretl Hendrickx, Tatyana Danyukova, Anke Baranowsky, Tim Rolvien, Alexandra Angermann, Michaela Schweizer, Johannes Keller, Jörg Schröder, Catherine Meyer-Schwesinger, Nicole Muschol, Chiara Paganini, Antonio Rossi, Michael Amling, Sandra Pohl, Thorsten Schinke Enzyme replacement therapy in mice lacking arylsulfatase B targets bone remodeling cells, but not chondrocytes published pages: , ISSN: 0964-6906, DOI: 10.1093/hmg/ddaa006 |
Human Molecular Genetics | 2020-02-13 |
2018 |
Sandra Pohl, Alexandra Angermann, Anke Jeschke, Gretl Hendrickx, Timur A Yorgan, Georgia Makrypidi-Fraune, Anita Steigert, Sonja C Kuehn, Tim Rolvien, Michaela Schweizer, Till Koehne, Mona Neven, Olga Winter, Renata Voltolini Velho, Joachim Albers, Thomas Streichert, Jan M Pestka, Christina Baldauf, Sandra Breyer, Ralf Stuecker, Nicole Muschol, Timothy M Cox, Paul Saftig, Chiara Paganini, Antonio Rossi, Michael Amling, Thomas Braulke, Thorsten Schinke The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover published pages: , ISSN: 0884-0431, DOI: 10.1002/jbmr.3563 |
Journal of Bone and Mineral Research | 2019-06-11 |
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The information about "LYSOBONE" are provided by the European Opendata Portal: CORDIS opendata.
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