Abstract

The goal of this Motor Neuron Disease (MND) Program Project Grant is to better understand MND. We plan to elucidate motor neuron-specific properties as well as factors that specifically cause motor neuron death. In some cases, there bay be a common pathway of damage with respect to the MNDs. There is an overall emphasis on genetically inherited MNDs. Some of the topics that we will focus on (e.g., damage from oxidative stress, and problems in DNA repair) may clarify non-MND neurodegenerative diseases. The individual projects are: Project 1 (Siddique)- Gene mapping studies in MN disorders; Project 2 (Roos/Miller)-Neurodegeneration and neuroprotection in MND; Project 3 (Woloschak)- The pathogenesis of MND in the wasted mouse; Core A-Clinical-scientific core that acts as a source of clinical information and specimens for members of the Program Project and involves experimental treatments, studies of sporadic and familial MND, and post-poliomyelitis syndrome; Core B- Administrative core. Our hope is that knowledge gained from the varied Projects in this grant may increase our understanding of human MND and lead to rational and effective treatments.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Program Projects (P01)
Project #
5P01NS021442-17
Application #
6393362
Study Section
Special Emphasis Panel (ZNS1-SRB-L (01))
Program Officer
Sheehy, Paul A
Project Start
1984-12-01
Project End
2003-05-31
Budget Start
2001-06-01
Budget End
2002-05-31
Support Year
17
Fiscal Year
2001
Total Cost
$975,174
Indirect Cost

  Institution

Name
University of Chicago
Department
Neurology
Type
Schools of Medicine
DUNS #
225410919
City
Chicago
State
IL
Country
United States
Zip Code
60637

  Publications

Thomas, Ajit G; Bodner, Amos; Ghadge, Ghanashyam et al. (2009) GCP II inhibition rescues neurons from gp120IIIB-induced neurotoxicity. J Neurovirol 15:449-57
Haley, Benjamin; Paunesku, Tatjana; Proti?, Miroslava et al. (2009) Response of heterogeneous ribonuclear proteins (hnRNP) to ionising radiation and their involvement in DNA damage repair. Int J Radiat Biol 85:643-55
Nix, W A; Berger, M M; Oberste, M S et al. (2004) Failure to detect enterovirus in the spinal cord of ALS patients using a sensitive RT-PCR method. Neurology 62:1372-7
Rezania, Kourosh; Yan, Jianhua; Dellefave, Lisa et al. (2003) A rare Cu/Zn superoxide dismutase mutation causing familial amyotrophic lateral sclerosis with variable age of onset, incomplete penetrance and a sensory neuropathy. Amyotroph Lateral Scler Other Motor Neuron Disord 4:162-6
Ghadge, Ghanashyam D; Slusher, Barbara S; Bodner, Amos et al. (2003) Glutamate carboxypeptidase II inhibition protects motor neurons from death in familial amyotrophic lateral sclerosis models. Proc Natl Acad Sci U S A 100:9554-9
Woloschak, Gayle E; Paunesku, Tatjana; Protic, Miroslava (2002) Sensitivity to low-level radiation in radiosensitive ""wasted"" mice. Mil Med 167:42-3
Lee, J P; Gerin, C; Bindokas, V P et al. (2002) No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis. J Neurochem 82:1229-38
Yang, Y; Hentati, A; Deng, H X et al. (2001) The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet 29:160-5
Hirano, M; Hung, W Y; Cole, N et al. (2000) Multiple transcripts of the human Cu,Zn superoxide dismutase gene. Biochem Biophys Res Commun 276:52-6
Hosler, B A; Siddique, T; Sapp, P C et al. (2000) Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA 284:1664-9

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