h a specific neurotransmitter receptor subunit (GABA A receptor alpha 1) rendered inactive exhibit the clinical characteristics of genetic essential tremor and respond to the same drugs as patients with essential tremor. This provides a consistent model in which to test novel potential treatments. An accompanying commentary by Joseph Jankovic and Jeffrey Noebels stresses that this new mutant mouse "provides an excellent model for tracing the developmental cascade of signaling changes that leads to a tremulous phenotype."
This paper represents the first direct demonstration that GABA A receptors help maintain proper motor function and provides evidence for the etiology of essential tremor involving loss of GABA A receptor alpha1 subunits. Alcohol withdrawal tremor is also associated with decreased expression of GABAA receptor alpha1 subunits.
TITLE: Genetic essential tremor in gamma-aminobutryric acid-A receptor alpha-1 subunit knockout mice
AUTHOR CONTACT:
A. Leslie Morrow
University of North Carolina School of Medicine, Chapel Hill, NC USA
Phone: 919-966-7682; Fax: 919-966-9099; E-mail: morrow@med.unc.edu
This article is available at: http://www.jci.org/cgi/content/full/115/3/774
ACCOMPANYING COMMENTARY:
TITLE: Genetic mouse models of essential tremor: are they essential?
AUTHOR CONTACT:
Joseph Jankovic
Baylor College of Medicine, Houston, TX USA
Phone: 713-798-5998; Fax: 713-798-6808; E-mail: josephj@bcm.tmc.edu
This article is available at: http://www.jci.org/cgi/content/full/115/3/584
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Contact: Stacie Bloom
press_releases@the-jci.org
212-342-4159
Journal of Clinical Investigation
1-Mar-2005
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