Since 2002 we have been investigating the field of NCL research. We want to understand what causes this fatal disease. Our ambitious aim is to develop a therapy in order to be able to cure NCL. Therefore, the € 50,000 NCL research award is awarded each year to projects that contribute to curing NCL. Furthermore we fund scholarships with our donations. Worldwide we are the biggest single sponsor in this area. To react quickly to new developments, we also offer short-term start-up support. These short projects can be extended to larger ones where appropriate. And because there are many overlaps between childhood and adult dementia, the Neurodegeneration Award was initiated this year.
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Postgraduate and research scholarships are awarded for up to three years. Regular reports and visits to laboratories are part of the programme. Candidates for funding can be recommended by their supervisors followed by an interview with our research leader. Here, two of them present their projects:
In Cooperation with Evotec, Daniela will test 2,000 compounds on a human CLN3 cell model and examine whether one of them can improve the condition of the defective neurons. Thanks to its infrastructure and many years of experience Evotec is the ideal partner to carry out high-throughput-screenings.
Supervisor: Dr. Guido Hermey, UKE, Hamburg
Funding Partners: Heinrich Hartmann Stiftung
Katarzyna studies how mitochondrial-lysosomal signaling differs in the absence of CLN3.The ultimate goal is to tease out metabolic consequences of CLN3-deficiency in the hope that this will generate new insights into perturbed pathways to be corrected and therapeutic approaches.
Supervisor: Dr. Nuno Raimundo, University Medical Center, Göttingen.
Funding Partners: dgroup, Hans Rosenthal Preis, HAORI-Stiftung Bremen, Heinz und Heide Dürr Stiftung, Online-Aktion von Julia Risel, von Poll GmbH.
Heart problems are a typical accompanying disease of NCL. In her research project, Emily is investigating cardiac muscle cells which host the NCL defect to find out their functionality.
Supervisor: Dr. Katja Rietdorf, The Open University, Milton Keynes
Funding Partner: Batten Research Fonds, Bijou Brigitte Stiftung, Böttcher-Stiftung, NCL-Golf-Trophy, RT4 golf tournament, Stichting Beat Batten
Gemma has successfully used the CRISPR/Cas method to produce hiPS cells with CLN3 mutations, which are similar to patients' cells but have the same genetic background. These cells form the basis for her studies and, have become important tools for other laboratories to test potential drugs.
Supervisor: Prof. Dr. Hans Schöler, Max Planck Institut Münster
Funding Partners: Batten Research Fonds, Berti Vogts, Münster Krimi Cup
Clinical-orientated projects are particularly prizeworthy. The prize money is used to fund postdoctoral fellows, to implement the submitted project and to promote young scientists. In order to find a cure for jNCL, scientists from all over the world are encouraged to submit their projects, especially those from related fields like Alzheimer's, ageing and lysosomal storage diseases. The last two awardees in brief:
Dr. Rebecca Ahrens-Nicklas (Children’s Hospital in Philadelphia, USA) received the 8th NCL Research Award. The aim of the project is to investigate the neural network changes that occur during disease progression in JNCL mouse models and assess their translational value. Ultimately, the goal is to assess the efficacy of therapeutics for their ability to normalize neural network function and translate findings to the clinic.
Funding Partner: Joachim Herz Stiftung.
Dr. Thomas Wishart and his PhD student Maica Llavero Hurtado (University of Edinburgh) were the winners of the 7th NCL research award. The objective of their research project is to identify new approaches for an effective NCL therapy with the help of the €50,000 prize money. Since there are indications that communications between NCL patients’ neurons are faulty, the team is focusing on neurons’ control centres, the synapses.
Funding Partner: ADM Hamburg AG, Online-Campaign "Save the Brain", HanseMerkur Recognition Award for Child Protection, Münster Krimi Cup
Thanks to the great support of the Joachim Herz Stiftung, we are able to announce another €100,000 grant. The so-called Neurodegeneration Award 2018 covers a postdoctoral fellowship salary for two years. The aim is to foster and improve synergies in resarch into NCL childhood dementia and age-related neurodegeneration. Therefore, applicants must meet the following requirements:
Ideally, postdoctoral candidates should conduct a considerable part of their work in both laboratories. Interested laboratories first send us a brief letter of intent (see download above). After acceptance you will be asked to send in a full proposal. Send the application form to firstname.lastname@example.org. If you need help to find a collaborating partner do not hesitate to contact us.
Funding proposals can also be requested for smaller projects. These projects shoud consist of research intended for practical application intended to form the basis of new projects.