RT Journal Article
SR Electronic
T1 Paroxysmal Dyskinesias in the Lethargic Mouse Mutant
JF The Journal of Neuroscience
JO J. Neurosci.
FD Society for Neuroscience
SP 8193
OP 8200
DO 10.1523/JNEUROSCI.22-18-08193.2002
VO 22
IS 18
A1 Zubair Khan
A1 H. A. Jinnah
YR 2002
UL http://www.jneurosci.org/content/22/18/8193.abstract
AB Lethargic mutant mice carry a mutation in the CCHB4gene, which encodes the β4 subunit of voltage-regulated calcium channels. These mutants have been shown to display a complex neurobehavioral phenotype that includes EEG discharges suggestive of absence epilepsy, chronic ataxia, and hypoactivity. The current studies demonstrate a fourth element of their phenotype, consisting of transient attacks of severe dyskinetic motor behavior. These attacks can be triggered by specific environmental and chemical influences, particularly those that stimulate locomotor activity. Behavioral and EEG analyses indicate that the attacks do not reflect motor epilepsy, but instead resemble a paroxysmal dyskinesia. The lethargic mutants provide additional evidence that calcium channelopathies can produce paroxysmal dyskinesias and provide a novel model for studying this unusual movement disorder.