Epilepsy in kcnj10 morphant zebrafish assessed with a novel method for long-term EEG recordings

PLoS ONE

Volumn 8, Issue 11, 2013, Pages

  Zdebik, Anselm A ^a   Mahmood, Fahad ^b   Stanescu, Horia C ^a   Kleta, Robert ^a,c   Bockenhauer, Detlef ^a,c   Russell, Claire ^b  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b ROYAL VETERINARY COLLEGE   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ANTICONVULSIVE AGENT; CONVULSANT AGENT; DIAZEPAM; INWARDLY RECTIFYING POTASSIUM CHANNEL; KCNJ10 (CHANNEL); PENTETRAZOLE;

ANIMAL; BRAIN; CHEMICALLY INDUCED; DISEASE MODEL; DRUG EFFECTS; ELECTROENCEPHALOGRAPHY; EPILEPSY; GENE SILENCING; GENETICS; PATHOPHYSIOLOGY; SEIZURES; ZEBRA FISH;

ANIMALS; ANTICONVULSANTS; BRAIN; CONVULSANTS; DIAZEPAM; DISEASE MODELS, ANIMAL; ELECTROENCEPHALOGRAPHY; EPILEPSY; GENE KNOCKDOWN TECHNIQUES; PENTYLENETETRAZOLE; POTASSIUM CHANNELS, INWARDLY RECTIFYING; SEIZURES; ZEBRAFISH;

EID: 84893954467     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0079765     Document Type: Article

References (20)

1. Bockenhauer D, Feather S, Stanescu HC, Bandulik S, Zdebik AA, et al. (2009) Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations. N Engl J Med 360: 1960-1970.
2. Olsen ML, Sontheimer H (2008) Functional implications for Kir4.1 channels in glial biology: from K+ buffering to cell differentiation. Journal of neurochemistry 107: 589-601.
3. Haj-Yasein NN, Jensen V, Vindedal GF, Gundersen GA, Klungland A, et al. (2011) Evidence that compromised K+ spatial buffering contributes to the epileptogenic effect of mutations in the human Kir4.1 gene (KCNJ10). Glia 59: 1635-1642.
4. Santoriello C, Zon LI (2012) Hooked! Modeling human disease in zebrafish. J Clin Invest 122: 2337-2343.
5. Baxendale S, Holdsworth CJ, Meza Santoscoy PL, Harrison MR, Fox J, et al. (2012) Identification of compounds with anti-convulsant properties in a zebrafish model of epileptic seizures. Dis Model Mech 5: 773-784.
6. Mahmood F, Mozere M, Zdebik AA, Stanescu HC, Tobin J, et al. (2013) Generation and validation of a zebrafish model of EAST (epilepsy, ataxia, sensorineural deafness and tubulopathy) syndrome. Dis Model Mech 6: 652-60
7. Baraban SC, Dinday MT, Castro PA, Chege S, Guyenet S, et al. (2007) A large-scale mutagenesis screen to identify seizure-resistant zebrafish. Epilepsia 48: 1151-1157. (Pubitemid 46854876)
8. Baraban SC, Taylor MR, Castro PA, Baier H (2005) Pentylenetetrazole induced changes in zebrafish behavior, neural activity and c-fos expression. Neuroscience 131: 759-768. (Pubitemid 40261229)
9. Bandulik S, Schmidt K, Bockenhauer D, Zdebik AA, Humberg E, et al. (2011) The salt-wasting phenotype of EAST syndrome, a disease with multifaceted symptoms linked to the KCNJ10 K+ channel. Pflugers Archiv : European journal of physiology 461: 423-435.
10. Freudenthal B, Kulaveerasingam D, Lingappa L, Shah MA, Brueton L, et al. (2011) KCNJ10 Mutations Disrupt Function in Patients with EAST Syndrome. Nephron Physiol 119: p40-p48.
11. Reichold M, Zdebik AA, Lieberer E, Rapedius M, Schmidt K, et al. (2010) KCNJ10 gene mutations causing EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) disrupt channel function. Proc Natl Acad Sci U S A 107: 14490-14495.
12. Thompson DA, Feather S, Stanescu HC, Freudenthal B, Zdebik AA, et al. (2011) Altered electroretinograms in patients with KCNJ10 mutations and EAST syndrome. J Physiol 589: 1681-1689.
13. Hortopan GA, Dinday MT, Baraban SC (2010) Spontaneous seizures and altered gene expression in GABA signaling pathways in a mind bomb mutant zebrafish. J Neurosci 30: 13718-13728.
14. Neusch C, Papadopoulos N, Muller M, Maletzki I, Winter SM, et al. (2006) Lack of the Kir4.1 channel subunit abolishes K+ buffering properties of astrocytes in the ventral respiratory group: impact on extracellular K+ regulation. Journal of neurophysiology 95: 1843-1852.
15. Pineda R, Beattie CE, Hall CW (2011) Recording the adult zebrafish cerebral field potential during pentylenetetrazole seizures. J Neurosci Methods 200: 20-28.
16. Bauzano-Poley E, Rodriguez-Barrionuevo AC (2001) [Electroencephalographic diagnosis of the idiopathic generalized epilepsies of childhood]. Rev Neurol 32: 365-372.
17. Westmoreland BF (1996) Epileptiform electroencephalographic patterns. Mayo Clin Proc 71: 501-511.
18. Buono RJ, Lohoff FW, Sander T, Sperling MR, O'Connor MJ, et al. (2004) Association between variation in the human KCNJ10 potassium ion channel gene and seizure susceptibility. Epilepsy Res 58: 175-183. (Pubitemid 38553953)
19. Ferraro TN, Golden GT, Smith GG, Martin JF, Lohoff FW, et al. (2004) Fine mapping of a seizure susceptibility locus on mouse Chromosome 1: nomination of Kcnj10 as a causative gene. Mammalian genome 15: 239-251. (Pubitemid 38453442)
20. Lenzen KP, Heils A, Lorenz S, Hempelmann A, Hofels S, et al. (2005) Supportive evidence for an allelic association of the human KCNJ10 potassium channel gene with idiopathic generalized epilepsy. Epilepsy Res 63: 113-118.