PMID: 38864Apr 1, 1979

An animal model for Huntington's disease

Biological Psychiatry
J T Coyle


In review is concerned with research done on an animal model for the hereditary neuropsychiatric disorder, Huntington's disease (HD). The neuropathology of HD involves primarily a selective degeneration of neurons with cell bodies in the striatum. Injection of kainic acid, a potent neuroexcitant structurally related to glutamic acid, into the rat striatum causes a selective neuronal degeneration resembling that of HD. Striatal cholinergic and GABAergic neurons, including their terminal projections in the substantia nigra, are affected by kainate; dopaminergic axons innervating the striatum as well as corticofugal fibers passing through the region are spared. The striatal kainate lesion has aided in the characterization of the neuronal circuitry in the nigrostriatal axis including the neuronal localization of dopamine-sensitive adenylate cyclase, neuroleptic binding sites, and GABA receptors. Studies in vivo and in vitro with kainate and its analogues suggest that the potent neurotoxicity of kainate involves a cooperative interaction between synaptically released glutamate and injected kainate on vulnerable neurons; prior destruction of cortico-striatal glutamatergic afferents attenuates kainate's neurotoxicity. The kainate mode...Continue Reading

Related Concepts

Lentiform Nucleus Structure
Nerve Degeneration
Glutamate Decarboxylase
Substantia Nigra Structure
Synaptic Receptors
Neural Pathways
Choline O-Acetyltransferase
Lupus Vasculitis, Central Nervous System
Gamma-Aminobutyric Acid, Zinc Salt (2:1)

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