Phenotypes for Cacna1a Tg(Pcp2-cre)2Mpin MGI:4949731 MGI Mouse

• dyskinesis is induced by stress or chemicals, such as a new environment, cage transport, short restraint, or administration of caffeine and alcohol

environmentally induced seizures ( J:170451 )

absence seizures ( J:170451 )

• mice exhibit sustained axial dystonia and repetitive clonic limb movements unlike wild-type mice

abnormal spike wave discharge ( J:170451 )

• mice exhibit stereotyped 6 to 7 spikes per second generalized spike-wave seizure discharges unlike wild-type mice

thin cerebellar granule layer ( J:170451 )

thin cerebellar molecular layer ( J:170451 )

small cerebellum ( J:170451 )

abnormal action potential ( J:170451 )

• at P21, only 2 of 30 cells exhibit spontaneous action potential activity compared with wild-type cells

• at P48, Purkinje cells lack spontaneous action potential firing unlike wild-type cells

• omegaAgalVA-treatment fails to reduced inhibitory postsynaptic current (IPSC) amplitudes unlike similarly treated wild-type mice

• however, response to omega CtxGVIA is normal

abnormal channel response ( J:170451 )

• Purkinje cells exhibit reduced P/Q-type channel current density compared with wild-type cells

ataxia ( J:170451 )

• mild to severe after 2 weeks of age

impaired coordination ( J:170451 )

• mice perform extremely poorly on a rotarod compared with wild-type mice

• mice exhibit impaired performance in incline and pole tests compared with wild-type mice

decreased grip strength ( J:170451 )

• mice exhibit impaired coordination of forepaw grip compared with wild-type mice