CHARACTERIZATION OF HEREDITARY CEREBELLAR ATAXIA

  • Werner, John S (PI)
  • Tolbert, Daniel (PI)
  • Byrnes, William (PI)
  • Southwick, C. (PI)
  • Schmidt, Steve (PI)
  • Copley, Shelley (PI)
  • Dufour, Darna (PI)
  • Barnes, Carol (PI)
  • Olson, Richard (PI)
  • McIntosh, J. (PI)
  • Johnston, Murray (PI)
  • Kuempel, Peter (PI)
  • Polson, Peter (PI)
  • van Gerven, Dennis (PI)
  • Bekoff, Anne (PI)
  • Gill, Stanley (PI)
  • Dynan, William (PI)
  • Snyder, Greg (PI)
  • Hedberg, Natalie (PI)
  • Mitton, Jeffrey (PI)
  • Sherwood, David (PI)
  • McNaughton, Bruce (PI)
  • Johnson, T. (PI)
  • McConkey, Edward (PI)
  • Fotino, Mircea (PI)
  • Ramirez, E. Fred (PI)
  • Gleeson, Todd (PI)
  • Rogers, Andrei (PI)
  • Eaton, Robert (PI)
  • Pardi, Arthur (PI)
  • McConkey, Edwin (PI)
  • Monson, Russel (PI)
  • Horii, Yoshiyuki (PI)
  • Kompala, Dhinaker (PI)
  • Maier, Steven (PI)
  • Miklowitz, David (PI)
  • Fulker, David (PI)
  • Kirkegaard, Karla (PI)
  • Duthcher, Susan (PI)
  • Bradshaw, Gary (PI)
  • Thomas, David (PI)
  • Koch, Tad (PI)
  • Wilson, J. (PI)
  • Stormo, Gary (PI)
  • Winey, Mark (PI)
  • Hanken, James (PI)
  • Kuchta, Robert (PI)
  • Parsons, Dorabeth (PI)
  • Kelso, A. (PI)
  • Landis, Clark (PI)
  • Melancon, Paul (PI)
  • Kinnamon, John (PI)
  • Rakowski-Dubois, M.A.R. (PI)
  • Greenwood, Michael (PI)
  • Boggiano, A.N.N. (PI)
  • Johnson, Thomas (PI)
  • Carlson, Lawrence (PI)
  • Healy, Alice (PI)
  • Wilson, James (PI)
  • Hand, Steven (PI)
  • Mazzeo, Robert (PI)
  • Wehner, Jeanne (PI)
  • Falke, Joseph (PI)
  • Milner-Brown, S. (PI)
  • Pickett-Heaps, J. (PI)
  • Kompala, Dhinakar (PI)
  • Dubin, Mark (PI)
  • Ekstrand, Bruce (PI)
  • Sievers, Robert (PI)

Project: Research project

Project Details

Description

Clinical signs of ataxia were noted in 3 male rats from a shipment of
50. Initial data suggested that the ataxia was primarily due to a
spontaneous, progressive Purkinje cell (PC) degeneration. The defect is
hereditary, appearing in the second generation of inbreeding affected
rats. The breeding colony has remained free of murine pathogens
including "rat parvovirus other". Purkinje cell degeneration in humans
has been associated with hereditary cerebellar ataxia, alcohol
intoxication, certain neoplasias, and aging. While PC degeneration has
been induced in rats, a spontaneous rat model of the disease is not
available. We propose a series of experiments to identify the temporal
and spatial features of PC degeneration in these affected rats and
correlate our findings with known human disease states. Our specific
aims are to: 1) identify the spatial distribution of degenerating PC's
in the cerebellum at different postnatal ages; 2) determine if PC
degeneration is localized to specific subpopulations of PC's; 3) survey
other areas of the CNS for neuronal degeneration; 4) determine if
degeneration of PC's results in alteration in the organization of
spinocerebellar afferent systems; and 5) determine if significant
differences exist between normal and affected rats in organs outside the
CNS.
StatusFinished
Effective start/end date4/1/799/29/93

Funding

  • National Institutes of Health

ASJC

  • Medicine(all)

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