Jacksonville - Mayo Clinic Jacksonville

Terrone Rosenberry, Ph.D.
(RG)  Interactions in the active site of acetylcholinesterase
$57,000.00	1/1/00 - 12/31/00	Year 3

Summary: Studies of the essential enzyme acetylcholinesterase will focus on its catalytic mechanism and on ways in which drug binding to its peripheral site alter catalytic activity.

Miami - University of Miami

John Barrett, Ph.D.
(RG)  Ca2+ regulation in motor neuron mitochondria 
in familial amyotrophic lateral sclerosis (FALS) mice
$53,382.00	1/1/00 - 12/31/00	Year 2
$54,660.00	1/1/01 - 12/31/01	Year 3

Summary: Researchers will test whether defects in mitochondrial uptake/extrusion of calcium contribute to early stages of motor neuron degeneration in a mouse model of FALS.

Lisa Baumbach, Ph.D.
(RG)  Continued identification of the X-linked 
lethal infantile SMA gene
$70,000.00	1/1/00 - 12/31/00	Year 1
$70,000.00	1/1/01 - 12/31/01	Year 2

Summary: The proposed study focuses on the identification of the casual disease gene for X-linked infantile SMA (XL-SMA) within the current candidate disease gene region of the X chromosome.

Walter Bradley, M.D.
(EMG)   Restricted funds for support of 
the Kessenich Family MDA/ALS Center
$420,000.00	4/1/99 - 3/31/00	Year 3

Murray P. Deutscher
(SG)  Miami Nature Biotechnology Winter Symposia
$5,000.00	2/1/00 - 2/28/00	Year 1

Summary: This symposium will allow researchers to discuss relationships of DNA and RNA and how these relationships may cause or be affected by disease.

Karl Magleby, Ph.D.
(RG)  Stimulation - Induced changes in transmitter release
$46,548.00	1/1/00 - 12/31/00	Year 3

Summary: This research will investigate the mechanism by which nerves release the transmitter substance that activates the contraction of muscles.

Carlos Moraes, Ph.D.
(RG)  Exploring compensatory mechanisms 
for mitochondrial tRNALys mutations
$50,000.00	7/1/99 - 6/30/00	Year 3

Summary: Researchers will study the molecular pathogenesis of a mitochondrial DNA mutation causing a severe and fatal neuromuscular disorder (MERRF) and attempt to compensate for the cellular defect by manipulating genes secondarily affected by the mutation.

(RG)  Analyzing and protecting CNS mitochondria 
in amyotrophic lateral sclerosis (ALS)
$69,799.00	7/1/00 - 6/30/01	Year 1
$72‚480.00	7/1/01 - 6/30/02	Year 2
$74‚697.00	7/1/02 - 6/30/03	Year 3

Summary: Mitochondria, the powerhouse of the cells, have been shown to have structural abnormalities in an animal model of ALS. Researchers plan to study mitochondrial function in these animals and create a new model that will facilitate the analysis of potentially beneficial drugs.

Richard L. Rotundo, Ph.D.
(RG)  Assembly of acetylcholinesterase and the 
synaptic basal lamina
$51,151.00	7/1/99 - 6/30/00	Year 1
$52,653.00	7/1/00 - 6/30/01	Year 2
$54,201.00	7/1/01 - 6/30/02	Year 3

Summary: Researchers are studying how acetylcholinesterase is targeted and retained at the neuromuscular synapse under normal conditions, and how it can be replaced when damaged or absent.

Ashok Verma, M.D., D.M.
(DG)  Mitochondrial role in amyotrophic lateral sclerosis (ALS)
$35,000.00	7/1/99 - 6/30/00	Year 1

Summary: Mitochondria are the cell powerhouse. A leading hypothesis implicates mitochondrial energy failure as a major mechanism in motor neuronal death in ALS. This hypothesis will be tested using modern tools of molecular genetics and cell physiology.

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