BAD knockout provides metabolic seizure resistance in a genetic model of epilepsy with sudden unexplained death in epilepsy

被引:13
|
作者
Foley, Jeannine [1 ]
Burnham, Veronica [1 ]
Tedoldi, Meghan [2 ]
Danial, Nika N. [2 ]
Yellen, Gary [1 ]
机构
[1] Harvard Med Sch, Dept Neurobiol, Boston, MA 02115 USA
[2] Harvard Med Sch, Dana Farber Canc Inst, Dept Canc Biol, Boston, MA USA
关键词
BAD; Kcna1(-/-); K(v)1.1; metabolic seizure resistance; KETOGENIC DIET; MICE; KV1.1;
D O I
10.1111/epi.13960
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Metabolic alteration, either through the ketogenic diet (KD) or by genetic alteration of the BAD protein, can produce seizure protection in acute chemoconvulsant models of epilepsy. To assess the seizure-protective role of knocking out (KO) the Bad gene in a chronic epilepsy model, we used the Kcna1(-/-) model of epilepsy, which displays progressively increased seizure severity and recapitulates the early death seen in sudden unexplained death in epilepsy (SUDEP). Beginning on postnatal day 24 (P24), we continuously video monitored Kcna1(-/-) and Kcna1(-/-) Bad(-/-) double knockout mice to assess survival and seizure severity. We found that Kcna1(-/-) Bad(-/-) mice outlived Kcna1(-/-) mice by approximately 2 weeks. Kcna1(-/-) Bad(-/-) mice also spent significantly less time in seizure than Kcna1(-/-) mice on P24 and the day of death, showing that Bad KO provides seizure resistance in a genetic model of chronic epilepsy.
引用
收藏
页码:E1 / E4
页数:4
相关论文
共 50 条
  • [31] Sudden unexplained death in epilepsy: Experience from clinical practice
    Jacobson, MP
    Schraeder, PL
    Leschek-Gelman, LM
    Vasoya, P
    EPILEPSIA, 1999, 40 : 231 - 231
  • [32] Do pets reduce the likelihood of sudden unexplained death in epilepsy?
    Terra, Vera C.
    Sakamoto, Americo C.
    Machado, Helio R.
    Martins, Luciana D.
    Cavalheiro, Esper A.
    Arida, Ricardo M.
    Stoellberger, Claudia
    Finsterer, Josef
    Scorza, Fulvio A.
    SEIZURE-EUROPEAN JOURNAL OF EPILEPSY, 2012, 21 (08): : 649 - 651
  • [33] Sudden unexplained death in epilepsy: An intracranially monitored case - Discussion
    Meldrum, B
    Simon
    Leppik
    Shorvon
    Nashef
    Walker
    Fish
    Bird
    Stephenson
    Brown
    Mameli
    EPILEPSIA, 1997, 38 : S57 - S59
  • [34] Cardiac arrhythmia and epilepsy genetic variants in sudden unexpected death in epilepsy
    Aschner, Amir
    Keller, Anne
    Williams, Andrew
    Whitney, Robyn
    Cunningham, Kris
    Hamilton, Robert M.
    Pollanen, Michael
    Donner, Elizabeth
    FRONTIERS IN NEUROLOGY, 2024, 15
  • [35] Genetic Basis of Sudden Unexpected Death in Epilepsy
    Bagnall, Richard D.
    Crompton, Douglas E.
    Semsarian, Christopher
    FRONTIERS IN NEUROLOGY, 2017, 8
  • [36] Evidence for Brainstem Network Disruption in a Focal Epilepsy and Sudden Unexplained Death in Epilepsy: Validation Study
    Goldman, Alica M.
    Mueller, Susanne G.
    Nei, Maromi M.
    Bateman, Lisa M.
    Friedman, Daniel
    Devinsky, Orrin
    Knowlton, Robert
    Laxer, Kenneth D.
    ANNALS OF NEUROLOGY, 2017, 82 : S161 - S161
  • [37] Rates of Status Epilepticus and Sudden Unexplained Death in Epilepsy in People With Genetic Developmental and Epileptic Encephalopathies
    Donnan, Alice M.
    Schneider, Amy L.
    Russ-Hall, Sophie
    Churilov, Leonid
    Scheffer, Ingrid E.
    NEUROLOGY, 2023, 100 (16) : E1712 - E1722
  • [38] PSYCHOACTIVE AGENTS, SEIZURE PRODUCTION, AND SUDDEN-DEATH IN EPILEPSY
    LIPKA, LJ
    LATHERS, CM
    JOURNAL OF CLINICAL PHARMACOLOGY, 1987, 27 (03): : 169 - 183
  • [39] Population-based study of the incidence of sudden unexplained death in epilepsy
    Ficker, DM
    So, EL
    Shen, WK
    Annegers, JF
    O'Brien, PC
    Cascino, GD
    Belau, PG
    NEUROLOGY, 1998, 51 (05) : 1270 - 1274
  • [40] THE SUDDEN UNEXPLAINED DEATH SYNDROME IN EPILEPSY - DEMOGRAPHIC, CLINICAL, AND POSTMORTEM FEATURES
    EARNEST, MP
    THOMAS, GE
    EDEN, RA
    HOSSACK, KF
    EPILEPSIA, 1992, 33 (02) : 310 - 316