Al. Gundlach et al., DEFICIT OF INHIBITORY GLYCINE RECEPTORS IN SPINAL-CORD FROM PERUVIAN PASOS - EVIDENCE FOR AN EQUINE FORM OF INHERITED MYOCLONUS, Brain research, 628(1-2), 1993, pp. 263-270
Inherited myoclonus in Poll Hereford calves and spasticity in the spas
tic mouse (spa/spa) are characterized by myoclonic jerks of the skelet
al musculature which occur spontaneously and in response to sensory st
imuli, symptoms resembling those in subconvulsive strychnine poisoning
. The primary, biochemical defect in these myoclonic animals is a defi
cit of inhibitory glycine receptors in the central nervous system. We
now report the occurrence of similar stimulus-induced myoclonus in ind
ividual, pure-bred Peruvian Paso horses and an associated, specific de
ficiency in the density of [H-3]strychnine binding to inhibitory glyci
ne receptors sites in spinal cord of these animals. Specificity of the
deficit was confirmed by a demonstrated lack of change in the density
of several other receptor types in affected spinal cord, including mu
scarinic receptors and GABA(A)/benzodiazepine receptors. In light of t
he existence of genetically-inherited myoclonus in other species, thes
e results suggest the occurrence of an equine form of the disorder.