Literature DB >> 27346881

Ketogenic diet treatment increases longevity in Kcna1-null mice, a model of sudden unexpected death in epilepsy.

Kristina A Simeone1, Stephanie A Matthews1, Jong M Rho2, Timothy A Simeone1.   

Abstract

Individuals with poorly controlled epilepsy have a higher risk for sudden unexpected death in epilepsy (SUDEP). With approximately one third of people with epilepsy not achieving adequate seizure control with current antiseizure drugs, there is a critical need to identify treatments that reduce risk factors for SUDEP. The Kcna1-null mutant mouse models risk factors and terminal events associated with SUDEP. In the current study, we demonstrate the progressive nature of epilepsy and sudden death in this model (mean age of mortality (± SEM), postnatal day [P] 42.8 ± 1.3) and tested the hypothesis that long-term treatment with the ketogenic diet (KD) will prolong the life of Kcna1-null mice. We found that the KD postpones disease progression by delaying the onset of severe seizures and increases the lifespan of these mutant mice by 47%. Future studies are needed to determine the mechanisms underlying the KD effects on longevity. Wiley Periodicals, Inc.
© 2016 International League Against Epilepsy.

Entities:  

Keywords:  Disease progression; Kv1.1 knockout; Mortality; Seizures; Survival

Mesh:

Substances:

Year:  2016        PMID: 27346881      PMCID: PMC4972642          DOI: 10.1111/epi.13444

Source DB:  PubMed          Journal:  Epilepsia        ISSN: 0013-9580            Impact factor:   5.864


  28 in total

1.  Kv1.1 potassium channel deficiency reveals brain-driven cardiac dysfunction as a candidate mechanism for sudden unexplained death in epilepsy.

Authors:  Edward Glasscock; Jong W Yoo; Tim T Chen; Tara L Klassen; Jeffrey L Noebels
Journal:  J Neurosci       Date:  2010-04-14       Impact factor: 6.167

2.  Incidence and mechanisms of cardiorespiratory arrests in epilepsy monitoring units (MORTEMUS): a retrospective study.

Authors:  Philippe Ryvlin; Lina Nashef; Samden D Lhatoo; Lisa M Bateman; Jonathan Bird; Andrew Bleasel; Paul Boon; Arielle Crespel; Barbara A Dworetzky; Hans Høgenhaven; Holger Lerche; Louis Maillard; Michael P Malter; Cecile Marchal; Jagarlapudi M K Murthy; Michael Nitsche; Ekaterina Pataraia; Terje Rabben; Sylvain Rheims; Bernard Sadzot; Andreas Schulze-Bonhage; Masud Seyal; Elson L So; Mark Spitz; Anna Szucs; Meng Tan; James X Tao; Torbjörn Tomson
Journal:  Lancet Neurol       Date:  2013-09-04       Impact factor: 44.182

Review 3.  Ketogenic diets, mitochondria, and neurological diseases.

Authors:  Lindsey B Gano; Manisha Patel; Jong M Rho
Journal:  J Lipid Res       Date:  2014-05-20       Impact factor: 5.922

Review 4.  Sudden unexpected death in epilepsy.

Authors:  Susan Duncan; Martin J Brodie
Journal:  Epilepsy Behav       Date:  2011-06-12       Impact factor: 2.937

5.  Protective effect of the ketogenic diet in Scn1a mutant mice.

Authors:  Stacey B B Dutton; Nikki T Sawyer; Franck Kalume; Patricia Jumbo-Lucioni; Karin Borges; William A Catterall; Andrew Escayg
Journal:  Epilepsia       Date:  2011-07-29       Impact factor: 5.864

6.  Sudden unexpected death in epilepsy: assessing the public health burden.

Authors:  David J Thurman; Dale C Hesdorffer; Jacqueline A French
Journal:  Epilepsia       Date:  2014-06-05       Impact factor: 5.864

7.  Ketogenic diet prevents epileptogenesis and disease progression in adult mice and rats.

Authors:  Theresa A Lusardi; Kiran K Akula; Shayla Q Coffman; David N Ruskin; Susan A Masino; Detlev Boison
Journal:  Neuropharmacology       Date:  2015-08-06       Impact factor: 5.250

8.  How does the ketogenic diet work? Four potential mechanisms.

Authors:  Nika N Danial; Adam L Hartman; Carl E Stafstrom; Liu Lin Thio
Journal:  J Child Neurol       Date:  2013-05-13       Impact factor: 1.987

9.  Structural consequences of Kcna1 gene deletion and transfer in the mouse hippocampus.

Authors:  H Jürgen Wenzel; Helene Vacher; Eliana Clark; James S Trimmer; Angela L Lee; Robert M Sapolsky; Bruce L Tempel; Philip A Schwartzkroin
Journal:  Epilepsia       Date:  2007-07-25       Impact factor: 5.864

10.  The ketogenic diet increases mitochondrial uncoupling protein levels and activity.

Authors:  Patrick G Sullivan; Nancy A Rippy; Kristina Dorenbos; Rachele C Concepcion; Aakash K Agarwal; Jong M Rho
Journal:  Ann Neurol       Date:  2004-04       Impact factor: 10.422

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  24 in total

Review 1.  New insights into the mechanisms of the ketogenic diet.

Authors:  Detlev Boison
Journal:  Curr Opin Neurol       Date:  2017-04       Impact factor: 5.710

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

Authors:  Jeannine Foley; Veronica Burnham; Meghan Tedoldi; Nika N Danial; Gary Yellen
Journal:  Epilepsia       Date:  2017-11-23       Impact factor: 5.864

3.  Ketogenic diet regulates the antioxidant catalase via the transcription factor PPARγ2.

Authors:  Sara Knowles; Sarah Budney; Malavika Deodhar; Stephanie A Matthews; Kristina A Simeone; Timothy A Simeone
Journal:  Epilepsy Res       Date:  2018-09-21       Impact factor: 3.045

4.  Scn2a deletion improves survival and brain-heart dynamics in the Kcna1-null mouse model of sudden unexpected death in epilepsy (SUDEP).

Authors:  Vikas Mishra; Bharat K Karumuri; Nicole M Gautier; Rui Liu; Timothy N Hutson; Stephanie L Vanhoof-Villalba; Ioannis Vlachos; Leonidas Iasemidis; Edward Glasscock
Journal:  Hum Mol Genet       Date:  2017-06-01       Impact factor: 6.150

Review 5.  Epigenetics and epilepsy prevention: The therapeutic potential of adenosine and metabolic therapies.

Authors:  Detlev Boison; Jong M Rho
Journal:  Neuropharmacology       Date:  2019-08-13       Impact factor: 5.250

Review 6.  Epilepsy and astrocyte energy metabolism.

Authors:  Detlev Boison; Christian Steinhäuser
Journal:  Glia       Date:  2017-10-17       Impact factor: 7.452

7.  Synergistic protection against acute flurothyl-induced seizures by adjuvant treatment of the ketogenic diet with the type 2 diabetes drug pioglitazone.

Authors:  Timothy A Simeone; Stephanie A Matthews; Kristina A Simeone
Journal:  Epilepsia       Date:  2017-05-28       Impact factor: 5.864

Review 8.  Ion Channels in Genetic Epilepsy: From Genes and Mechanisms to Disease-Targeted Therapies.

Authors:  Julia Oyrer; Snezana Maljevic; Ingrid E Scheffer; Samuel F Berkovic; Steven Petrou; Christopher A Reid
Journal:  Pharmacol Rev       Date:  2018-01       Impact factor: 25.468

9.  Regulation of brain PPARgamma2 contributes to ketogenic diet anti-seizure efficacy.

Authors:  Timothy A Simeone; Stephanie A Matthews; Kaeli K Samson; Kristina A Simeone
Journal:  Exp Neurol       Date:  2016-08-12       Impact factor: 5.330

10.  Neuron-specific Kv1.1 deficiency is sufficient to cause epilepsy, premature death, and cardiorespiratory dysregulation.

Authors:  Krystle Trosclair; Hemangini A Dhaibar; Nicole M Gautier; Vikas Mishra; Edward Glasscock
Journal:  Neurobiol Dis       Date:  2020-01-21       Impact factor: 5.996

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