PUBLICATION

Emerging epilepsy models: insights from mice, flies, worms and fish

Authors
Baraban, S.C.
ID
ZDB-PUB-070330-1
Date
2007
Source
Current Opinion in Neurology   20(2): 164-168 (Review)
Registered Authors
Baraban, Scott
Keywords
epilepsy, invertebrate, model, organism, seizure
MeSH Terms
  • Animals
  • Diptera
  • Disease Models, Animal*
  • Drug Evaluation, Preclinical
  • Epilepsy/genetics*
  • Epilepsy/physiopathology
  • Fishes
  • Humans
  • Mice
  • Nematoda
PubMed
17351486 Full text @ Curr. Opin. Neurol.
Abstract
PURPOSE OF REVIEW: Animal models provide a means to investigate fundamental mechanisms of abnormal electrical discharge (i.e., seizures). Understanding the pathogenesis of epilepsy and therapy development have greatly benefited from these models. Here we review recent mouse mutants featuring spontaneous seizures and simpler organisms. RECENT FINDINGS: New genetically engineered mice provide additional insights to cellular mechanisms underlying seizure generation (BK calcium-activated potassium channels and interneuron-expressed sodium channels), genetic interactions that exacerbate seizure phenotype (Scn2a, Kcnq2 and background) and neurodevelopmental influences (Dlx transcription factors). Mutants for neuronal nicotinic acetylcholine receptors, Glut-1 deficiency and aquaporin channels highlight additional seizure phenotypes in mice. Additional models in Caenorhabditis elegans (Lis-1) and Danio rerio (pentylenetetrazole) highlight a reductionist approach. Taking further advantage of 'simple' organisms, antiepileptic drugs and genetic modifiers of seizure activity are being uncovered in Drosophila. SUMMARY: Studies of epilepsy in mutant mice provide a framework for understanding critical features of the brain that regulate excitability. These, and as yet undiscovered, mouse mutants will continue to serve as the foundation for basic epilepsy research. Interestingly, an even greater potential for analyzing epileptic phenotypes may lie in the more widespread use of genetically tractable organisms such as worms, flies and zebrafish.
Genes / Markers
Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping