Kevin Talbot, MB, University of Oxford, Oxford, England, Funding: 1 Year Dr. Talbot of Oxford University will study patients with the rare neurodegenerative disorder primary lateral sclerosis (PLS) using new magnetic resonance imaging technologies developed in Oxford. This will allow an assessment of the progress of the disease and, it is hoped, will allow trials of treatments in PLS patients. PLS is a form of motor neuron disease, related to ALS (Amyotrophic Lateral Sclerosis or Lou Gehrig's Disease) but with a number of atypical features, including slower progression and absence of muscle wasting. Its cause is unknown and there are currently no treatments.

Dr. Teepu Siddique of Northwestern University has been awarded a research grant for the study of primary lateral sclerosis (PLS) entitled “Molecular Genetic Study of Primary Lateral Sclerosis”. Dr. Siddique identified the first gene known to cause primary lateral sclerosis (PLS) in children, and with collaboration, developed the first transgenic ALS mouse model in 1994. Additionally, Siddique and collaborators discovered the first gene for ALS1 in 1993, and the gene for juvenile inherited ALS (ALS2) in 2001. More than one gene is involved in the development of PLS, however, and Siddique and his laboratory group currently are investigating families with PLS not linked to the same locus.

Dr. John K. Fink of the University of Michigan has been awarded a research grant for the study of primary lateral sclerosis (PLS) entitled “Molecular Basis of Primary Lateral Sclerosis: Search for Alsin Binding Factors”. Dr. Fink’s project will study the relationship between the proteins responsible for juvenile PLS and hereditary spastic paraplegias (HSP), a very similar and often miss-diagnosed disorder.
In 2006 Dr. Fink, M.D. was awarded a two-year grant totaling $120,000 for his outstanding proposal entitled Developing treatment for childhood onset hereditary spastic paraplegia (SPG3A HSP).