Phenotypes Associated with This Genotype

Genotype
MGI:3796547

• Allelic Composition: Gna11^tm1Soff/Gna11^tm1Soff; Gnaq^tm2Soff/Gnaq^tm2Soff; Tg(Camk2a-cre)1Gsc/0
• Genetic Background: involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * FVB/N

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:111559 )

• age-dependent reduction in survival due to tonic-clonic seizures

nervous system

seizures ( J:111559 )

• around 3 months of age, mice show spontaneous epileptic seizures; animals affected and seizure number per animal increase with age

• mutants show reduced latency to seizure onset and tendency to have stronger seizures when treated with kainic acid or PTZ than wild-type; seizure-induced lethality is greatly increased

• blockade of cannabinoid receptors does not result in aggravation of kainic acid-induced seizures in mutants but does in wild-type controls

• treatment of mutants with endocannabinoid reuptake inhibitor causes a significant amelioration of kainic acid induced seizures

clonic seizures ( J:111559 )

• most seizures are myoclonic

tonic-clonic seizures ( J:111559 )

• some animals exhibit tonic-clonic seizures with age

gliosis ( J:111559 )

• older mice display reactive gliosis in CA1 region of hippocampus

hippocampal neuron degeneration ( J:111559 )

• older animals particularly those with symptomatic epilepsy show neuronal degeneration in CA1 region

abnormal nervous system electrophysiology ( J:123222 )

• no CCK4-induced (cholecystokinin receptor₂ agonist) current is detected in amygdala slices while inward current response in wild-type is unaffected

behavior/neurological

seizures ( J:111559 )

• around 3 months of age, mice show spontaneous epileptic seizures; animals affected and seizure number per animal increase with age

• mutants show reduced latency to seizure onset and tendency to have stronger seizures when treated with kainic acid or PTZ than wild-type; seizure-induced lethality is greatly increased

• blockade of cannabinoid receptors does not result in aggravation of kainic acid-induced seizures in mutants but does in wild-type controls

• treatment of mutants with endocannabinoid reuptake inhibitor causes a significant amelioration of kainic acid induced seizures

clonic seizures ( J:111559 )

• most seizures are myoclonic

tonic-clonic seizures ( J:111559 )

• some animals exhibit tonic-clonic seizures with age

cellular

gliosis ( J:111559 )

• older mice display reactive gliosis in CA1 region of hippocampus