QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

HOME  |   SEARCH  |   ARCHIVE  |   SUBSCRIBE  |   CONTACT  |   HELP

This Article Full Text Full Text (PDF) Submit an eLetter Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via ISI Web of Science (7) Citing Articles via Google Scholar Google Scholar Articles by Khan, Z. Articles by Jinnah, H. A. Search for Related Content PubMed PubMed Citation Articles by Khan, Z. Articles by Jinnah, H. A.

 Previous Article  |  Next Article 

The Journal of Neuroscience, September 15, 2002, 22(18):8193-8200

Paroxysmal Dyskinesias in the Lethargic Mouse Mutant

Zubair Khan and H. A. Jinnah

Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland 21287

Lethargic mutant mice carry a mutation in the CCHB4 gene, which encodes the 4 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.

Key words: lethargic; mouse mutant; calcium channel; channelopathy; paroxysmal dyskinesia; movement disorder; epilepsy

---

Copyright © 2002 Society for Neuroscience  0270-6474/02/22188193-08$05.00/0

This article has been cited by other articles:

				E. Glasscock and M. A. Tanouye Drosophila couch potato Mutants Exhibit Complex Neurological Abnormalities Including Epilepsy Phenotypes Genetics, April 1, 2005; 169(4): 2137 - 2149. [Abstract] [Full Text] [PDF]

Home  |   Search  |   Archive  |   Subscribe  |   Contact  |   Help