• Folstein, Susan (PI)
  • Coyle, Joseph (PI)
  • Kazazian, Haig (PI)
  • Snyder, Solomon (PI)
  • Zee, David (PI)
  • Folstein, Marshal F. (PI)
  • Borchelt, David (PI)
  • Hurko, Orest (PI)
  • Rosenblatt, Adam (PI)
  • Reading, Sarah (PI)
  • Troncoso, Juan (PI)
  • Margolis, Russell (PI)
  • Ranen, Neal (PI)
  • Wagster, Molly V. (PI)
  • Hedreen, John (PI)
  • Strauss, Milton (PI)
  • Stine, O. (PI)
  • Delong, Mahlon (PI)
  • Whitehouse, Peter (PI)
  • Irani, David (PI)
  • Ranen, Neal (PI)
  • Stine, O. (PI)
  • Wagster, Molly V. (PI)
  • Aylward, Elizabeth (PI)
  • Brandt, Jason (PI)
  • Ross, Christopher (PI)

Project: Research project

Project Details


The Baltimore Huntington's Disease Project (BHDP) is a cohesive research
program, which includes research on etiology, pathogenesis, and treatment.
In this application, we request funding for years 11-15. Using a variety of new methods, we propose to study the mechanism of gene
action in HD. Dr. Orest Hurko (Mitochondrial Function) approaches the
question through Dr. Joseph Coyle (Selective Neuronal Vulnerability) uses
the rodent HD model and neuronal culture to investigate the basic
mechanisms leading to glutamate-induced excitotoxicity and will test drugs
in these systems for their ability to prevent neuronal damage. One outcome
of this work is a separately funded Experimental Therapeutic Trial of
Idebenone. A related theme is the role of extra-striatal pathology in HD. Drs. Chris
Ross and John Hedreen (Extra-Striatal Pathology) use neuropathological and
neurochemical experiments to elucidate the pathogenetic meaning of cortical
and amygdala pathology in HD. Dr. Jason Brandt (Correlates of Frontal-
Striatal Degeneration) uses neuropsychological methods to assess the
significance of cortical atrophy (using MRI) in cognitive and functional
deficits. Dr. David Zee (Ocular Motor Function), uses novel paradigms for
testing ocular motor function in HD patients to study the anatomy and
function of ocular motor pathways and their changes in HD. The BHDP is coordinated through its Cores, and through the physical
proximity of the investigators whose offices and laboratories are in the
Meyer Building. The Administrative Core provides both formal and informal
forums for discussion of ideas and progress, and provides statistical
services for all Projects and Cores. The Clinical Core recruits and
maintains approximately 300 well-characterized HD patients. A separately
funded research project, Presymptomatic Testing for HD, grew out of the
resources of the Clinical Core. The Pathology Core has brain material on
over 70 HD patients, most of whom were followed until death by the Clinical
Core. Progress will be reviewed by an External Review Committee. Our in-house
Ethics Committee reviews questions relating to patient participation in
Effective start/end date7/1/804/30/12


  • National Institutes of Health: $987,297.00


  • Medicine(all)
  • Neuroscience(all)


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