A single gene error of noradrenergic axon growth synchronizes central neurones (original) (raw)
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- Published: 02 August 1984
Nature volume 310, pages 409–411 (1984)Cite this article
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Abstract
One strategy for deciphering inherited neurological disease is to examine the expression of individual genes controlling the assembly and physiology of specific cell groups within the developing mammalian central nervous system (CNS). This neurogenetic approach, using defined single-locus mutations arising on coisogeneic mouse strains, has recently been used to analyse a major class of neuronal membrane diseases involving abnormal excitability, the epilepsies, and to identify examples of hereditary variation in signalling properties at central synapses1. An interesting mutation, the Tottering (tg) gene2, causes a delayed onset, recessive neurological disorder in the mouse featuring a stereotyped triad of ataxia, intermittent myoclonus and cortical spike-wave discharges accompanied by behavioural absence seizures which resemble petit mal epilepsy3. Axon branches of the locus coeruleus, a noradrenergic brain-stem nucleus, hyperinnervate specific target regions of the tg brain4. The number of parent coerulean perikarya is unaffected, indicating a true proliferation of the terminal axonal arbor. With the exception of this unusually precise error of axonal growth, no other cytopathology has been identified in the tg brain. Here I present evidence that selective lesions of the central noradrenergic axons early in development limit the expression of the disease.
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Authors and Affiliations
- Departments of Neuroscience and Neuropathology, Children's Hospital Medical Center and Harvard Medical School, Boston, Massachusetts, 02115, USA
Jeffrey L. Noebels
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- Jeffrey L. Noebels
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Noebels, J. A single gene error of noradrenergic axon growth synchronizes central neurones.Nature 310, 409–411 (1984). https://doi.org/10.1038/310409a0
- Received: 18 October 1983
- Accepted: 04 May 1984
- Issue Date: 02 August 1984
- DOI: https://doi.org/10.1038/310409a0