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Citation
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HERO ID
7705234
Reference Type
Journal Article
Title
A single gene error of noradrenergic axon growth synchronizes central neurones
Author(s)
Noebels, JL
Year
1984
Is Peer Reviewed?
1
Journal
Nature
ISSN:
0028-0836
EISSN:
1476-4687
Volume
310
Issue
5976
Page Numbers
409-411
Language
English
PMID
6462226
DOI
10.1038/310409a0
Web of Science Id
WOS:A1984TC54400044
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 synapses. An interesting mutation, the Tottering (tg) gene, 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 epilepsy. Axon branches of the locus coeruleus, a noradrenergic brain-stem nucleus, hyperinnervate specific target regions of the tg brain. 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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