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Title: Disorder of the inhibitory glycine receptor: inherited myoclonus in Poll Hereford calves.
Austin Authors: Gundlach, Andrew L
Affiliation: University of Melbourne, Department of Medicine, Austin Hospital, Heidelberg, Victoria, Australia
Issue Date: 1-Jul-1990
Publication information: Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology; 4(10): 2761-6
Abstract: Inherited congenital myoclonus in Poll Hereford calves is characterized by hyperesthesia and myoclonic jerks of the skeletal musculature that occur spontaneously and in response to sensory stimuli. The symptoms of the disorder suggest a failure of spinal inhibition and are similar to those in subconvulsive strychnine poisoning. Strychnine is a high-affinity antagonist of the synaptic actions of glycine. Our recent biochemical studies revealed a specific and marked deficit in [3H]strychnine binding sites in brain stem and spinal cord membranes from myoclonic calves compared with unaffected controls, reflecting a decrease in inhibitory glycine receptors. Glycine is a major inhibitory neurotransmitter in the mammalian central nervous system, and glycinergic transmission is important for the control of both motor and sensory functions in the spinal cord. In other studies, synaptosomes prepared from affected spinal cord showed a significantly increased ability to accumulate [3H]glycine, indicating an increased capacity of the high-affinity neuronal reuptake system for glycine. In contrast, spinal cord glycine concentrations and stimulus-induced release of endogenous glycine, measured in vitro, were unaltered. The major clinical signs of this myoclonic disorder can be explained by the reported deficiency of inhibitory glycine receptors in brain stem and spinal cord, and future research will be directed toward identifying the nature of the genetic alteration responsible for this deficiency. The characteristics of this bovine receptor abnormality are similar to those described for the mutant spastic mouse.
Gov't Doc #: 2165010
Journal: FASEB journal : official publication of the Federation of American Societies for Experimental Biology
Type: Journal Article
Subjects: Animals
Cattle Diseases
Receptors, GABA-A.physiology
Receptors, Glycine
Receptors, Neurotransmitter.genetics.metabolism
Appears in Collections:Journal articles

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