RESEARCH GRANTS AWARDED BY THE BDFA

SPRING 2008/2009 

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 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 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.  

BDFA Grant Awards Call 1

The following grants were awarded by the BDFA after the review of the applications received in April 2008: 

Dr. Jon Cooper, King’s College London /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, King’s College London & University College London ICH /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, University College London /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, University College London / Golgi conference  £270

This was granted to Sara Mole to support her attendance at a conference in Italy at which international scientists would be discussing membrane trafficking.  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.  

BDFA Grant Awards Call 2

Round 1

The  following grants were awarded after the review of the first round of applications received in December 2008: 

Dr. Jon Cooper, King’s College London / PhD studentship Tuition fees £5,000

For Tuition Fees for a new PhD student starting in 2009 who will be working on “Pathogenic impact of immune-related cells in two models of Batten Disease.” This project will study the effects of immune cells in the brain which, in Batten Disease, leave the blood and enter the brain at the same time as brain cells start to die. It is possible that this directly contributes to the loss of brain cells and this will be investigated using mouse models of infantile and juvenile Batten Disease. Understanding the extent and nature of immune cells in the brain may lead to new ways to combat the disease.

Dr. Sara Mole, University College London / Continued investigation of the large vacuole phenotype associated with loss of Btn1p function £5,000

To continue her Lysosome studies: looking at how and when the gene for CLN3 is prevented from working, the Lysosomes within the cell increase in size. Further knowledge about this gene will help in the overall understanding of the disease and could potentially lead to new forms of therapy.

We also allocated €5k in sponsorship of the international NCL 2009 meeting in Hamburg in June to support this important event for the NCL scientific and patient community.   

Round 2 

The following grants were awarded after the review of the second round of applications received in April 2009: 

Dr. Jon Cooper, King’s College London / NCL 2009 Travel cost support £1,100

To support the costs of Thomas Kühl, Sarah Ahmadi, Angelika Saje and Natalie Masento members of Dr Jon Cooper’s lab to attend the NCL2009 conference in Hamburg. This is part of our continuing policy to encourage, and support young researchers in the NCL field to build a sound base for research in the future.

Dr. Sara Mole, University College London /EDLSD conference £500

To attend the EDLSD conference in Sept 09. This is an important opportunity for her to present her latest data, increase knowledge and raise Batten Disease awareness at this European LSD group scientific meeting. A report on the relevant research presented will be produced for the BDFA.

Dr. Sara Mole, University College London / Investigation of Golgi morphology £4,000

To provide the laboratory consumables of a high-calibre MRC Studentship student, Jennifer Winter, in Sara’s lab for one of her rotation subjects. This will enable a much needed project in Batten disease to be undertaken with the prospect of her undertaking a future Batten’s-related PhD project.