BDSRA Research Awards

BDSRA Research Awards

BDSRA awards research grants August 2000.

BDSRA Awards Research Grants

On August 5, 2000 at the Annual Conference, BDSRA awarded 14 research grants totaling $ 234,000.

Michael Bennett, Ph.D., University of Texas Southwestern - $15,000
“Function of CLN3 Protein in Non-Neuronal Cells” - This project is to identify the protein associated with Juvenile Batten Disease by examination of Pancreatic Islet cells and other non-neuronal cells.

Quynh Chu-LaGraff, Ph.D. Union College - $ 6900
“Development of a Drosophila Model for NCLs” - This project is to develop a fruit fly model of NCLs, thus adding to the models that can be studied and used in other research projects.

Jonathan Cooper, Ph.D. - King’s College, London, England - $9300
“Comparative Anatomical Studies in the Neuronal Ceroid Lipofuscinoses” - Funds to be used towards purchase of a confocal microscope that will be use in anatomical studies of the NCLs.

Beverly Davidson, Ph.D., University of Iowa - $2560
“Cell and Gene replacement in NCL” - Funds to be used to house 92 mice for gene therapy studies.

Outi Heinonen, Ph.D. - National Public Health Institute - Finland - $14,000
“Significance of Palmitoyl Protein Thioesterase for Development, Maturation and Death of Neurons” Project is determine why cortical and retinal neurons are the first and most affected cells types in Infantile NCL.

Martin Katz, Ph.D. - University of Missouri - $23,000
“Further Development and Characterization of a Mouse Gene Knock-Out Model for Classic Late-infantile NCL”. Project is to complete development of a mouse model for Late Infantile Batten Disease.

Frederick J. Marshall, M.D., University of Rochester - $30,000
“Development of a Unified Batten Disease Rating Scale” Project is to develop a means of measuring Batten Disease in order to be prepared for eventual human trials.

David Pearce, Ph.D. - University of Rochester - $13,500
“Phenotypic Reversal of the Neurodegenerative Effects of Batten Disease in CLN3 and mnd mice” Project is to study means of reversing effects of Batten Disease in the CLN3 and mnd mice models.

Leena Peltonen, M.D., Ph.D. - UCLA - $25,000
“Properties of the CLN5 Polypeptide and it’s Collaboration with Other Neuronal Ceroid Lipofuscinosis Proteins”. Project is to identify the protein associated with CLN5 and it’s location and to determine whether it has a relationship with other NCL associated proteins.

A. N. Siakotos, Ph.D. - Indiana University - $35,000
“A Therapeutic Strategy to Prevent Decline of Cognitive Function in NCL Patients”. Project is to study the effects of certain dietary supplements to prevent decline of cognitive functions.

Steven Walkley, DVM, Ph.D. - Albert Einstein College of Medicine - $20,000
“Mechanisms of Selective Neuronal Vulnerability in Batten Disease”. Project is to determine why neurons in some areas of the brain die in massive numbers while neurons in other areas of the brain survive.

Ruth Wheeler, Ph.D. - University College London - $1700
“Molecular Cloning of the Gene for a Variant Form of Late-Infantile NCL”. Funds to be used to purchase new computer system that will aid in identification of gene for Variant Late Infantile - CLN6.

Nanbert Zhong, M.D. - Institute for Basic Research - $3000
“Clinical Enzymatic assays on CLN1-encoded PPT1 for INCL” Funding is to establish enzyme activity assay for Infantile Batten Disease.

Nanbert Zhong, M.D. - Institute for Basic Research - $35,000
“Identification and Characterization of NCLs Families by Molecular Genetic Testing”. Funding to be used to continue the identification of point mutations of the known NCL genes and for genetic testing of family members.”

These research grants were possible through the tremendous efforts of many fund raising events and through the kindness and generosity of many people’s donations. The Canadian Chapter proved the research awards for Drs. Cooper, Heinonen, and Wheeler.

For additional information contact BDSRA at 1-800-448-4570 or 740-927-4298 outside USA and Canada.

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