title: Connecting Sarcomere Protein Mutations to Pathogenesis in Cardiomyopathies: The Development of "Disease in a Dish" Models.
abstract: Recent technological and protocol developments have greatly increased the ability to utilize stem cells transformed into cardiomyocytes as models to study human heart muscle development and how this is affected by disease associated mutations in a variety of sarcomere proteins. In this perspective we provide an overview of these emerging technologies and how they are being used to create better models of "disease in a dish" for both research and screening assays. We also consider the value of these assays as models to explore the seminal processes in initiation of the disease development and the possibility of early interventions.
authors.affiliation: Department of Bioengineering, University of Washington Seattle, WA, USA.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Zaunbrecher
authors.affiliation: Department of Bioengineering, University of WashingtonSeattle, WA, USA; Center for Cardiovascular BiologySeattle, WA, USA; Institute for Stem Cell and Regenerative Medicine, University of WashingtonSeattle, WA, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Regnier
keywords: cardiomyopathies; contraction; gene editing; methods development; stem cell derived cardiomyocytes