Minneapolis - University of Minnesota

Ewa Prochniewicz, Ph.D.
(DG) Dynamics of actin's interaction with myosin or dystrophin
$ 35,000.00	7/1/99 - 6/30/00	Year 2
$ 35,000.00	7/1/00 - 6/30/01	Year 3

Summary: Spectroscopy and microscopy will be used to analyze the molecule interactions of the protein actin with two other key muscle proteins -- myosin and dystrophin.

Laura Ranum, Ph.D.
(RG) Genetic and clinical characterization of myotonic dystrophy (MMD) type 2
$ 60,000.00	1/1/00 - 12/31/00	Year 2
$ 60,000.00	1/1/01 - 12/31/01	Year 3

Summary: Researchers propose a series of studies aimed at understanding the specific DNA changes that can lead to genetically distinct forms of MMD. Understanding what, at the molecular level, is common to the various forms of this disorder will help us better understand these diseases.

Osha Roopnarine, Ph.D.
(RG) Function and structural dynamics of myosin mutations
$ 59,720.00	7/1/99 - 6/30/00	Year 1
$ 64,549.00	7/1/00 - 6/30/01	Year 2
$ 65,646.00	7/1/01 - 6/30/02	Year 3

Summary: Researchers will perform biochemical, mechanical, spectroscopic and microscopic analysis of myosin mutations to assess the molecular basis for muscle disease.

David Thomas, Ph.D.
(RG) Site-directed spectroscopic probes of myosin
$ 49,613.00	7/1/99 - 6/30/00	Year 3

Summary: Researchers will study the motions and orientations of the light chain domain of the myosin head, with the ultimate goal of understanding the mechanism of muscle contraction and its abnormalities.

Rochester - Mayo Clinic

Andrew Engel, M.D.
(RG) Congenital myasthenic syndromes
$ 70,077.00	1/1/00 - 12/31/00	Year 3

Summary: Congenital myasthenias will be studied by a multifaceted approach to gain better insights into pathogenesis, diagnosis, treatment, and prevention.

Grazia Isaya, M.D., Ph.D.
(RG) Role of mitochondrial intermediate peptidase in Friedreich's ataxia (FA)
$ 32,586.00	7/1/99 - 6/30/00	Year 2

Summary: Researchers propose to construct genetically-designed mutant mice to investigate whether defects in the mitochondrial protein import machinery contribute to the development of FA.

Ann Reed, M.D.
(RG) Peptide receptivity of the JDMS molecule HLA-DQA1*0501
$ 50,000.00	7/1/98 - 3/30/00	Extended

Summary: Researchers will investigate the peptide binding of the molecules responsible for an immune response and the triggering of disease onset in juvenile dermatomyositis.

St. Paul - University of Minnesota

Bianca M. Conti-Fine, M.D.
(RG) Anti-nicotinic receptor T cells in ocular myasthenia gravis (MG)
$ 50,000.00	7/1/99 - 6/30/00	Year 1
$ 50,000.00	7/1/00 - 6/30/01	Year 2

Summary: Researchers will study autoimmune CD4+T cells and antibodies in patients with ocular and generalized MG, to understand why the symptoms remain localized to the ocular muscle in ocular myasthenia, and spread to all muscles in generalized myasthenia.

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