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 presented in 2018.
For questions on the subjects of research and development or funding please contact Dr. Herman van der Putten.
A compilation of all currently funded research projects can be found in our annual review.
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:
Lysosomal calcium ion channels may be an important target in CLN3 disease. Sukanya Arcot Kannabiran is studying this channel function at the microdomain level using high-resolution microscopes. The results could be important for both childhood and senile dementia.
Supervisor: Prof. Andreas Guse, UKE Hamburg- Eppendorf, GER
Funding Partners: Bijou Brigitte Stiftung, „Hand in Hand für Norddeutschland“ (NDR), Peter Jensen Stiftung.
Masood Ahmad Wani is investigating which pathological changes affect signal transmission between neurons in the CLN3 brain. He has already found very early changes in the NCL model that occur even before the deposits develop.
Supervisor: Dr. Benedikt Grünewald, University Medical Center Mainz, GER
Funding Partners: Helga und Alfred Buchwald Stiftung, Reinhard Frank-Stiftung, Scheck Stiftung, Stiftung Bostelmann, von Poll Immobilien.
Prof. Alessia Calcagnì's project at Baylor College of Medicine in Houston, Texas, USA, focuses on the role of microglial cells in CLN3 disease. Microglial cells
are the immune cells of the brain. CLN3 patients and also corresponding mouse models show pathological changes in microglial cells.
Collaborating partners are Dr. Paolo Grumati and Dr. Davide Cacchiarelli (both TIGEM, Pozzuoli, Italy) as well as Prof. Monther Abu-Remaileh (Stanford University, CA, USA).
We thank the Werner Reichenberger Stiftung for funding this project.
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 13th NCL Research Award 2023 was given to Dr. Alessandro Ori and Dr. Julia C. Heiby from the Leibniz Institute on Aging - Fritz Lipmann Institute in Jena, Germany, for their project "Targeting alterations of the lysosomal proteome in Batten disease".
In NCL, there is a pathological change in the protein composition of the lysosomes, the “recycling yards“ of the cells. The entire set of proteins in cells is called proteome. The aim of the research work is to reproduce these changes in a suitable in vitro model in order to be able to test therapeutic approaches.
Funding Partner: Joachim Herz Stiftung.
Thanks to the great support of the Joachim Herz Stiftung, we were 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. Two laboratories from the above-mentioned fields must apply jointly. The postdoctoral fellow should thus have the opportunity to conduct research in both laboratories to ensure the exchange of respective expertise.
The "Neurodegeneration Research Award" 2018, endowed with 100,000 euros, goes to two neuroscientists from Munich and Boston: Sabina Tahirovic, from the German Center for Neurodegenerative Diseases e.V. (DZNE) and Susan Cotman, from Massachusetts General Hospital, will use the prize money to research commonalities between dementia in children and adults.
The focus of the two-year project are the immune cells of the brain. For all their differences, childhood dementias share a number of similarities with Alzheimer's disease. These include the activation of the brain's immune cells, known as microglia. As a result, chronic inflammation develops. The scientists want to understand how the microglia change in childhood dementia and why they enter a state of permanent activation.
On this basis, new targets for therapy are to be found, which could also be explored with regard to the treatment of Alzheimer's disease.
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.
Because childhood dementia NCL is so rare, many doctors are not familiar with the disease. The consequences are numerous misdiagnoses and a diagnosis time of two to four years.
An agonising time of uncertainty for the children and families. Furthermore, an early diagnosis is also of great importance for further family planning, as NCL is a genetic disease. That is why
we are using various measures to specifically raise awareness among doctors and students.
So we organize symposia together with NCL experts at medical congresses and give lectures at clinics and in specialist circles. We also distribute NCL leaflets for ophthalmologists and paediatricians.