Deficit of spinal cord glycine/strychnine receptors in inherited myoclonus of Poll Hereford calves

Inherited myoclonus of Poll Hereford calves is characterized by hyperesthesia and myoclonic jerks of the skeletal musculature, which occur spontaneously and in response to sensory stimuli. The disease shows autosomal recessive inheritance, and significant proportions of the Poll Hereford herds in ma...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1988-09, Vol.241 (4874), p.1807-1810
Main Authors: GUNDLACH, A. L, DODD, P. R, GRABARA, C. S. G, WATSON, W. E. J, JOHNSTON, G. A. R, HARPER, P. A. W, DENNIS, J. A, HEALY, P. J
Format: Article
Language:English
Subjects:
Amino acids
Amino Acids - metabolism
Animals
Biological and medical sciences
breeds
Calves
Cattle
Cattle Diseases - genetics
Cattle Diseases - physiopathology
Classical genetics, quantitative genetics, hybrids
Diseases
estricnina
Fundamental and applied biological sciences. Psychology
genetic disorders
Genetic inheritance
Genetics
Genetics of eukaryotes. Biological and molecular evolution
glicina
Glycine
Glycine receptors
Hereford cattle
Medical research
medula espinal
Membrane proteins
moelle epiniere
Muscle spasticity
Muscular system
Myoclonus
Myoclonus - genetics
Myoclonus - physiopathology
Myoclonus - veterinary
Neurotransmitter receptors
Polls
race
razas
Receptors
Receptors, Glycine
Receptors, Neurotransmitter - deficiency
Spinal cord
Spinal Cord - physiopathology
strychnine
Synaptosomes
ternero
trastornos geneticos
trouble genetique
veau
Vertebrata
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Summary:Inherited myoclonus of Poll Hereford calves is characterized by hyperesthesia and myoclonic jerks of the skeletal musculature, which occur spontaneously and in response to sensory stimuli. The disease shows autosomal recessive inheritance, and significant proportions of the Poll Hereford herds in many countries are thought to be carriers of the mutant gene. Studies revealed a specific and marked (90 to 95 percent) deficit in [3H]strychnine binding sites in spinal cord membranes from myoclonic animals compared to controls, reflecting a loss of, or defect in, glycine/strychnine receptors. Spinal cord synaptosomes prepared from affected animals showed a significantly increased ability to accumulate [3H]glycine, indicating an increased capacity of the high-affinity neuronal uptake system for glycine. In contrast, stimulus-induced glycine release and spinal cord glycine concentrations were unaltered.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.2845573