Abstract

The focus of this Program Project is the motor nerve cell and its susceptibility to disease. The Program combines direct studies of patients with amyotrophic lateral sclerosis (ALS) and basic in vitro tissue culture and in vivo animal studies related to motor neuron biology and pathology. The cental resource is a large clinic devoted to the care and study of ALS patients. Both sporadic and familial forms of ALS are represented. Specific areas of study in this Program include defining properties of motor nerve cells and the neuromuscular junction in health and disease using immunochemical, molecular biologic and physiologic techniques; characterizing muscle and glial cell-derived motor neuron trophic factors, determining the bases of neuronal viral tropism, and evaluating the feasibility of transplantation of motor nerve cells.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Program Projects (P01)
Project #
5P01NS021442-02
Application #
3099856
Study Section
Neurological Disorders Program Project Review A Committee (NSPA)
Project Start
1984-12-01
Project End
1987-11-30
Budget Start
1985-12-01
Budget End
1986-11-30
Support Year
2
Fiscal Year
1986
Total Cost
Indirect Cost

  Institution

Name
University of Chicago
Department
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

Showing the most recent 10 out of 38 publications