Mapt deletion fails to rescue premature lethality in two models of sodium channel epilepsy.
Ann Clin Transl Neurol
; 5(8): 982-987, 2018 Aug.
Article
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| MEDLINE
| ID: mdl-30128323
ABSTRACT
Deletion of Mapt, encoding the microtubule-binding protein Tau, prevents disease in multiple genetic models of hyperexcitability. To investigate whether the effect of Tau depletion is generalizable across multiple sodium channel gene-linked models of epilepsy, we examined the Scn1b-/- mouse model of Dravet syndrome, and the Scn8aN1768D/+ model of Early Infantile Epileptic Encephalopathy. Both models display severe seizures and early mortality. We found no prolongation of survival between Scn1b-/-,Mapt+/+ , Scn1b-/-,Mapt+/-, or Scn1b-/-,Mapt-/- mice or between Scn8aN1768D/+,Mapt+/+ , Scn8aN1768D/+,Mapt+/- , or Scn8aN1768D/+,Mapt-/- mice. Thus, the effect of Mapt deletion on mortality in epileptic encephalopathy models is gene specific and provides further mechanistic insight.
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MEDLINE
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En
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Ann Clin Transl Neurol
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2018
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Article