About Batten Disease

Contact

BDFA Professionals

Downloads

BDFA award new Research Grants 

The BDFA’s Vision is to bring light to Batten Disease by being the central point of excellence in the UK for supporting affected families and to facilitate research into the disease.” In support of our Mission Statement the BDFA awards grants through our new Research Grants Application and Approval procedures to facilitate scientific mechanisms, potential cures and treatments for Batten Disease/Neuronal Ceroid Lipofusinoses (NCL).  

It is a very exciting time in the research field and the more we can support the scientists in their efforts to find out more about Batten Disease, the closer we will come to finding viable therapies. Our new Policy involves a multi-tiered approach with each Tier relating to the size of amount to be awarded and the type of research that the grant would support. Award levels are subject to available funds and it is hoped that these will significantly increase in the future. Briefly, these are: Tier 1 up to £500; Tier 2 greater than £500 up to £5,000; Tier 3 greater than £5,000. The following grants were awarded after the review of the first round of applications received in April 2008: 

Dr. Jon Cooper

/Studentship Fees for BDSRA-funded student Catherine Kielar  £5,000

The grant is for studies being performed by a highly-talented PhD student in Jon’s lab on the study of the vulnerable cells in the Thalamus (brain cells), which are known to start dying surprisingly early in the disease. This only happens after the brain’s immune system becomes activated and the synapses used by the brain cells to talk to one another become disorganized and are then lost. Catherine is looking at how and why these events happen as a first step towards blocking them, which may open the way for novel forms of therapy for Batten disease. 

Dr. Jon Cooper & Dr. Hannah Mitchison

/Investigating enhanced Neurogenesis in Batten disease  £5,000

This exciting new project involves looking at the growth of new brain cells. Although, cells are dying in the Batten disease brain, it appears that the brain is trying to repair itself by making new brain cells. For this to happen we will need to understand how and why this is taking place and then this might be something we can take advantage of to provide a new form of therapy. 

Dr. Sara Mole

/Lysosome studies  £5,000

For this study, an experienced research scientist in Sara’s lab will be looking at Lysosomes (these are the part of the cells which degrade and recycle unwanted components). When the gene CLN3 that causes Juvenile BD is prevented from working, the Lysosome gets bigger. This project will investigate why this happens and if any other cell parts are altered. This will significantly contribute to the understanding of this gene and could potentially identify new targets for therapy.  

Dr. Sara Mole will be attending the “The Golgi Meeting: Membrane trafficking in global cellular responses” in Italy in September supported by funds from the BDFA awarded under Tier 1. Sara’s recent research, into CLN3/BD, has taken an unexpected direction into a new area. Attending this high profile conference will enable her to present her latest work, increase her knowledge and explore new collaborations whilst raising the profile of BD to international experts who may not have previously heard about it.