Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
5R01NS032214-10
Application #
6639452
Study Section
Special Emphasis Panel (ZNS1-SRB-R (01))
Program Officer
Gwinn, Katrina
Project Start
1994-08-01
Project End
2006-04-30
Budget Start
2003-05-01
Budget End
2004-04-30
Support Year
10
Fiscal Year
2003
Total Cost
$318,000
Indirect Cost
Name
Thomas Jefferson University
Department
Biochemistry
Type
Schools of Medicine
DUNS #
053284659
City
Philadelphia
State
PA
Country
United States
Zip Code
19107
Yersak, Jill M; Montie, Heather L; Chevalier-Larsen, Erica S et al. (2017) The 11S Proteasomal Activator REG? Impacts Polyglutamine-Expanded Androgen Receptor Aggregation and Motor Neuron Viability through Distinct Mechanisms. Front Mol Neurosci 10:159
Berger, Tamar R; Montie, Heather L; Jain, Pranav et al. (2015) Identification of novel polyglutamine-expanded aggregation species in spinal and bulbar muscular atrophy. Brain Res 1628:254-264
Pratt, William B; Gestwicki, Jason E; Osawa, Yoichi et al. (2015) Targeting Hsp90/Hsp70-based protein quality control for treatment of adult onset neurodegenerative diseases. Annu Rev Pharmacol Toxicol 55:353-71
Chua, Jason P; Reddy, Satya L; Yu, Zhigang et al. (2015) Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor-mediated disease. J Clin Invest 125:831-45
Heine, Erin M; Berger, Tamar R; Pluciennik, Anna et al. (2015) Proteasome-mediated proteolysis of the polyglutamine-expanded androgen receptor is a late event in spinal and bulbar muscular atrophy (SBMA) pathogenesis. J Biol Chem 290:12572-84
Pratt, William B; Morishima, Yoshihiro; Gestwicki, Jason E et al. (2014) A model in which heat shock protein 90 targets protein-folding clefts: rationale for a new approach to neuroprotective treatment of protein folding diseases. Exp Biol Med (Maywood) 239:1405-13
Chua, Jason P; Reddy, Satya L; Merry, Diane E et al. (2014) Transcriptional activation of TFEB/ZKSCAN3 target genes underlies enhanced autophagy in spinobulbar muscular atrophy. Hum Mol Genet 23:1376-86
Wang, Adrienne M; Miyata, Yoshinari; Klinedinst, Susan et al. (2013) Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation. Nat Chem Biol 9:112-8
Cooper, Lori J; Merry, Diane E (2013) Cell biological approaches to investigate polyglutamine-expanded AR metabolism. Methods Mol Biol 1017:241-53
Chevalier-Larsen, Erica S; Merry, Diane E (2012) Testosterone treatment fails to accelerate disease in a transgenic mouse model of spinal and bulbar muscular atrophy. Dis Model Mech 5:141-5

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