Open AccessCiliary neurotrophic factor protects striatal output neurons in an animal model of Huntington disease.
Author(s) -
Keith D. Anderson,
Nikos Panayotatos,
Thomas L. Corcoran,
Ronald M. Lindsay,
Stanley J. Wiegand
Publication year - 1996
Publication title -
proceedings of the national academy of sciences of the united states of america
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.011
H-Index - 771
eISSN - 1091-6490
pISSN - 0027-8424
DOI - 10.1073/pnas.93.14.7346
Subject(s) - ciliary neurotrophic factor , neurotrophic factors , huntington's disease , neurotrophin , neuroscience , quinolinic acid , nerve growth factor , brain derived neurotrophic factor , glutamate receptor , glial cell line derived neurotrophic factor , neurodegeneration , huntingtin , biology , medicine , disease , receptor , tryptophan , biochemistry , amino acid
Huntington disease is a dominantly inherited, untreatable neurological disorder featuring a progressive loss of striatal output neurons that results in dyskinesia, cognitive decline, and, ultimately, death. Neurotrophic factors have recently been shown to be protective in several animal models of neurodegenerative disease, raising the possibility that such substances might also sustain the survival of compromised striatal output neurons. We determined whether intracerebral administration of brain-derived neurotrophic factor, nerve growth factor, neurotrophin-3, or ciliary neurotrophic factor could protect striatal output neurons in a rodent model of Huntington disease. Whereas treatment with brain-derived neurotrophic factor, nerve growth factor, or neurotrophin-3 provided no protection of striatal output neurons from death induced by intrastriatal injection of quinolinic acid, an N-methyl-D-aspartate glutamate receptor agonist, treatment with ciliary neurotrophic factor afforded marked protection against this neurodegenerative insult.