Audiogenic Seizures in Eleven Mouse Strains

Fuller, John L.; Sjursen, Frank H.

doi:10.1093/oxfordjournals.jhered.a107565

Cited by 101 publications

(39 citation statements)

References 10 publications

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“…stimulus assay (6,11). Previous studies indicate that adult mice on a pure B6 background are more resistant to seizure induction than other strains, such as 129; however, juvenile mice have not been investigated (33). In our pilot experiments using the audiogenic stimulus assay at PND 25, we found that 60% of wildtype mice on a 129 background exhibited debilitating tonic-clonic seizures that led to death.…”

Section: As Mice Display An Altered Developmental Time Course Of Ultrmentioning

confidence: 81%

Seizure-like activity in a juvenile Angelman syndrome mouse model is attenuated by reducing Arc expression

Mandel‐Brehm

Salogiannis

Dhamne

et al. 2015

Proc. Natl. Acad. Sci. U.S.A.

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Angelman syndrome (AS) is a neurodevelopmental disorder arising from loss-of-function mutations in the maternally inherited copy of the UBE3A gene, and is characterized by an absence of speech, excessive laughter, cognitive delay, motor deficits, and seizures. Despite the fact that the symptoms of AS occur in early childhood, behavioral characterization of AS mouse models has focused primarily on adult phenotypes. In this report we describe juvenile behaviors in AS mice that are strain-independent and clinically relevant. We find that young AS mice, compared with their wild-type littermates, produce an increased number of ultrasonic vocalizations. In addition, young AS mice have defects in motor coordination, as well as abnormal brain activity that results in an enhanced seizure-like response to an audiogenic challenge. The enhanced seizure-like activity, but not the increased ultrasonic vocalizations or motor deficits, is rescued in juvenile AS mice by genetically reducing the expression level of the activity-regulated cytoskeleton-associated protein, Arc. These findings suggest that therapeutic interventions that reduce the level of Arc expression have the potential to reverse the seizures associated with AS. In addition, the identification of aberrant behaviors in young AS mice may provide clues regarding the neural circuit defects that occur in AS and ultimately allow new approaches for treating this disorder.Angelman syndrome | ARC | EPHEXIN5 | ultrasonic vocalizations | EEG

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Section: As Mice Display An Altered Developmental Time Course Of Ultrmentioning

confidence: 81%

Seizure-like activity in a juvenile Angelman syndrome mouse model is attenuated by reducing Arc expression

Mandel‐Brehm

Salogiannis

Dhamne

et al. 2015

Proc. Natl. Acad. Sci. U.S.A.

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“…Susceptibility to audiogenic seizures (AS) varies widely between inbred strains of mice (2,3). The genetic basis for the difference in susceptibility to AS between DBA and C57BL mice has been studied several times since the strain difference was discovered by Hall (1).…”

Section: Introductionmentioning

confidence: 99%

Genetic dissection of susceptibility to audiogenic seizures in inbred mice.

Neumann

Collins

1991

Proc. Natl. Acad. Sci. U.S.A.

107

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Mice of some inbred strains, such as 21-dayold DBA/2J mice, have generalized convulsions when exposed to intense auditory stimulation. Analysis of susceptibility to audiogenic seizures in BXD recombinant inbred strains has demonstrated the influence of at least three loci. One locus, Asp-], is located on chromosome 12 between Ah and D12Nyul; another locus, Asp-2, is on chromosome 4, tightly linked to b.Here we report evidence that Asp-2 is located within an 8-centimorgan segment distal to b and that Asp-3 is linked to Mtv-1 on chromosome 7. We also present evidence that these three loci account for most of the heritable variation in susceptibility to audiogenic seizures in crosses of DBA/2J and C57BL/6J mice and that susceptibility to audiogenic seizures is influenced by genomic imprinting. Thus, genomic imprinting may complicate linkage and mapping studies and should be considered in analyses of complex modes of inheritance.Convulsive seizures can be induced in experimental animals by intense auditory stimuli (1, 2). Susceptibility to audiogenic seizures (AS) varies widely between inbred strains of mice (2,3). The genetic basis for the difference in susceptibility to AS between DBA and C57BL mice has been studied several times since the strain difference was discovered by Hall (1). The typical AS consists of wild running followed by a clonic seizure and then a tonic seizure that is usually fatal, unless the mouse is resuscitated (1, 2, 4). DBA/2J (D2) mice are susceptible from 14 to 42 days of age with peak sensitivity at 21 days (5), whereas C57BL/6J (B6) mice are resistant to intense auditory stimuli. Variation in AS susceptibility has been attributed to a single locus (6-8), two loci (9, 10), and multifactorial (or polygenic) modes of inheritance (11)(12)(13)(14). The differing conclusions of these investigators can be attributed to many causes, including the use of different DBA and C57BL sublines, mice of different ages, and different experimental protocols (4, 15, 16).Testing of the BXD series of recombinant inbred (RI) strains and F1 hybrids from crosses between BXD RI strains and D2 revealed that at least three loci are involved in the genetic variation in AS susceptibility in crosses between D2 and B6 mice (14). Two of these loci have already been given chromosomal assignments. Asp-] (audiogenic seizure prone-1, formerly Ias) is tightly linked to the Ah locus on chromosome 12 (13,14,17), and Asp-2 (formerly asp) is located on chromosome 4 (7, 8), tightly linked to b (brown) (14).Here we report the mapping of Asp-3 by a reevaluation of published data (8,13,14,(17)(18)(19) in the light of advances in our understanding of the mode of inheritance of susceptibility to AS in crosses of D2 and B6 mice. We also report the results of reciprocal backcrosses that confirm the conclusions of the genetic dissection. MATERIALS AND METHODSThe data from two multipoint crosses reported by Collins (8) were recompiled and reexamined in an attempt to place Asp-2 on the linkage map (see Tables 1 and 2). In the firs...

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“…The F 1 hybrid which has an intermediate seizure susceptibility (8) also had an intermediate choline transport capacity. Audiogenic seizure proneness appears to be an autosomal recessive (1,2). More extensive cross breeding studies would be needed to determine whether this was linked to cholinergic transport capacity.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, the induction of convulsions, either by pharmacological or audiogenic means, is more readily accomplished in the DBA/2J line than in C57BL/ 6J mice (1). This differential sensitivity is maximal at 21 days age when the convulsive proneness of OBA mice is greatest (2,3).…”

Section: Introductionmentioning

confidence: 99%

Seizure proneness and neurotransmitter uptake

1979

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The ability of midbrain homogenates from two strains of mice to accumulate several putative neurotransmitters, or their precursor in the case of acetylcholine, has been examined. The high-affinity transport mechanisms toward glutamate. GABA, dopamine, and glycine were similar in both strains. The seizure-prone DBA/2IBG strain had a significantly higher capacity to transport choline than did the relatively seizure-resistant C57BL/6 IBG mice. However, no difference in the density of muscarinic binding sites in the two mouse strains was found.

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Audiogenic Seizures in Eleven Mouse Strains

Cited by 101 publications

References 10 publications

Seizure-like activity in a juvenile Angelman syndrome mouse model is attenuated by reducing Arc expression

Seizure-like activity in a juvenile Angelman syndrome mouse model is attenuated by reducing Arc expression

Genetic dissection of susceptibility to audiogenic seizures in inbred mice.

Seizure proneness and neurotransmitter uptake

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