title: Audiogenic seizure susceptibility in thyroid hormone receptor beta-deficient mice.
abstract: As early-onset hypothyroidism produces audiogenic seizure susceptibility (AGS) in rodents, the role of TR alpha 1 and TR beta thyroid hormone receptors in AGS was investigated. AGS occurs in mice lacking specifically TR beta (Thrb(tm1/tm1)) and is marked by early onset and persistence, thereby differing from mouse strains where AGS is age-restricted. Thrb(tm1/tm1) mice display AGS whether on a mixed 129/Sv x C57BL/6J or congenic C57BL/6J background. 27% of wild-type mice on the mixed and 0% on the congenic background exhibited AGS. The inability of Thrb(tm1/tm1) mice to downregulate the response to sustained acoustic stimulation may reside in the brain or in the auditory system itself as Thrb(tm1/tm1) mice also display auditory deficits. The AGS phenotype identifies a novel neurological role for TR beta.
authors.affiliation: Department of Human Genetics, Mount Sinai School of Medicine, 1425 Madison Avenue, New York, NY 10029, USA.
authors.forename: L
authors.initials: L
authors.lastname: Ng
authors.forename: P E
authors.initials: PE
authors.lastname: Pedraza
authors.forename: J S
authors.initials: JS
authors.lastname: Faris
authors.forename: B
authors.initials: B
authors.lastname: Vennström
authors.forename: T
authors.initials: T
authors.lastname: Curran
authors.forename: G
authors.initials: G
authors.lastname: Morreale de Escobar
authors.forename: D
authors.initials: D
authors.lastname: Forrest
mesh_terms: D017668:Age of Onset; D000818:Animals; D001306:Auditory Pathways / Q000503:physiopathology*; D020195:Epilepsy, Reflex / Q000503:physiopathology*; D007037:Hypothyroidism / Q000503:physiopathology; D051379:Mice; D020297:Mice, Congenic; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D011988:Receptors, Thyroid Hormone / Q000235:genetics* / Q000378:metabolism*
chemical_list: D011988:Receptors, Thyroid Hormone