Knockin animal models of inherited arrhythmogenic diseases: what have we learned from them?

J Cardiovasc Electrophysiol. 2007 Sep;18(10):1117-25. doi: 10.1111/j.1540-8167.2007.00884.x. Epub 2007 Jun 16.

Abstract

Mouse models are becoming an increasingly accepted method of studying human diseases. Knockin and knockout techniques have several advantages over traditional transgenic overexpression, and the versatility of the knockin mouse allows the study of both gain of function mutations through targeted mutagenesis, as well as the replacement of one gene by another functional gene. Here, we will review the methods available to generate knockin mice; provide an overview of the techniques used to study electrophysiology in the mice at the cellular, organ, and whole animal level; and highlight knockin mice that have implications for inherited arrhythmias. Specifically, we will focus on models that used knockin mice to clarify gene expression, identify similarities and differences between related genes, and model human arrhythmia syndromes. Our goal is to provide the reader with a general understanding of studies done on knockin mouse models of inherited arrhythmias as well as ideas for future directions.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Animals, Genetically Modified / genetics*
  • Disease Models, Animal*
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / genetics*
  • Humans
  • Mice
  • Mice, Transgenic