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Vol. 27, No. 5, 2005
Issue release date: 2005
Section title: Original Paper
Dev Neurosci 2005;27:321–332
(DOI:10.1159/000086712)

Auditory Deficits Associated with the Frings Mgr1 (Mass1) Mutation in Mice

Klein B.D. · Fu Y.-H. · Ptacek L.J. · White H.S.
Departments of aPharmacology and Toxicology, bNeurobiology and Anatomy, cNeurology and Human Genetics, Howard Hughes Medical Institute, and dAnticonvulsant Drug Development Program, University of Utah, Salt Lake City, Utah, USA

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 10/18/2004
Accepted: 2/8/2005
Published online: 9/5/2005

Number of Print Pages: 12
Number of Figures: 3
Number of Tables: 4

ISSN: 0378-5866 (Print)
eISSN: 1421-9859 (Online)

For additional information: http://www.karger.com/DNE

Abstract

The gene responsible for the audiogenic seizure (AGS) phenotype in Frings mice, which was identified and originally designated Mass1, is now referred to as Mgr1. Although the function of the gene product is not known, the expression pattern suggests a role in the developing CNS. Hearing impairment is often observed in AGS-susceptible rodents and is thought to contribute to the pathology of AGS. We therefore hypothesized that the Frings mouse exhibits early-onset hearing impairment and that the Frings Mgr1 mutation is responsible for the hearing impairment phenotype that leads to the development of AGS susceptibility. Auditory brainstem response (ABR) thresholds were used to evaluate auditory function in mice carrying the Frings Mgr1 allele and were compared with other AGS-susceptible and -resistant mice. ABR testing demonstrated that mice possessing the Frings Mgr1 allele exhibit a mild to moderate level of hearing impairment that is present during the days following hearing onset. Furthermore, the hearing impairment resulting from the Frings Mgr1 allele is relatively stable, which explains the long duration of AGS susceptibility exhibited by Frings mice compared with other AGS-susceptible mice.


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 10/18/2004
Accepted: 2/8/2005
Published online: 9/5/2005

Number of Print Pages: 12
Number of Figures: 3
Number of Tables: 4

ISSN: 0378-5866 (Print)
eISSN: 1421-9859 (Online)

For additional information: http://www.karger.com/DNE


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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