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Phenotypes Associated with This Genotype
Genotype
MGI:3836256
Allelic
Composition
Cacna1atm3Maag/Cacna1atm3Maag
Genetic
Background
B6.129P2-Cacna1atm3Maag
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cacna1atm3Maag mutation (0 available); any Cacna1a mutation (66 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Neurological deficits after spreading depression in Cacna1atm1Maag/Cacna1atm1Maag and Cacna1atm3Maag/Cacna1atm3Maag mice

mortality/aging
• immediately after a seizure that occurs 45 to 75 minutes following a KCl-induced single spreading depression, 2 of 3 mice die due to respiratory arrest unlike similarly treated wild-type mice

behavior/neurological
• some mice remain unconscious for up to 10 minutes following a single spreading depression induced by KCl unlike wild-type mice
• topical application of 300 mM KCl induces a single spreading depression that causes hemiplegia, leaning, circling, unconsciousness for up to 10 minutes, seizures 45 to 75 minute after treatment, and, in 2 of 3 mice, death due to respiratory arrest unlike in similarly treated wild-type mice
• following a single spreading depression, mice are unable to move along a wire and fall more often than wild-type mice
• induction of multiple spreading depressions with KCl leads to more severe motor deficits than in wild-type mice
• following induction of multiple spreading depressions with KCl, female mice exhibit more severe and longer lasting motor deficits than male mice and unlike in wild-type mice
• a single spreading depression induced by KCl causes hemiplegia with leaning and circling unlike in wild-type mice
• a single spreading depression induced by KCl causes hemiplegia with leaning and circling unlike in wild-type mice
• a single spreading depression induced by KCl causes hemiplegia with leaning and circling unlike in wild-type mice
• 45 to 75 minutes following a single spreading depression induced by KCl unlike in simialrly treated wild-type mice

nervous system
• 45 to 75 minutes following a single spreading depression induced by KCl unlike in simialrly treated wild-type mice
• following induction with KCl, spreading depression frequency and propagation speed are increased, especially in females, compared to in wild-type mice and Cacna1atm1Maag homozygotes
• ovariectomy and cessation of estrous cycling eliminates sex differences
• KCl-induced cortical spreading depressions propagate into the striatum unlike in wild-type mice with greater frequency and shorter latencies than in Cacna1atm1Maag homozygotes
• corticaostriatal propagation of KCl-induced cortical spreading depressions is greater in female mice than in male mice
• mice exhibit 1 or more recurrent spreading depressions following brief topical KCl application and extensive washing unlike in wild-type mice

respiratory system
• immediately after a seizure 45 to 75 minutes following a KCl-induced single spreading depression, 2 of 3 mice die due to respiratory arrest unlike similarly treated wild-type mice

homeostasis/metabolism
• topical application of 300 mM KCl induces a single spreading depression that causes hemiplegia, leaning, circling, unconsciousness for up to 10 minutes, seizures 45 to 75 minute after treatment, and, in 2 of 3 mice, death due to respiratory arrest unlike in similarly treated wild-type mice
• KCl-induced cortical spreading depressions propagate into the striatum unlike in wild-type mice with greater frequency and shorter latencies than in Cacna1atm1Maag homozygotes
• corticaostriatal propagation of KCl-induced cortical spreading depressions is greater in female mice than in male mice
• mice exhibit 1 or more recurrent spreading depressions following brief topical KCl application and extensive washing unlike in wild-type mice
• immediately after a seizure that occurs 45 to 75 minutes following a KCl-induced single spreading depression, 2 of 3 mice die due to respiratory arrest unlike similarly treated wild-type mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Migraine, Familial Hemiplegic, 1; FHM1 141500 J:144701


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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory