Scientists have created functioning human heart tissue that exhibits Barth syndrome (Image: Shutterstock)

The researchers started by taking skin cells from two patients afflicted with Barth syndrome, an inherited and currently-untreatable heart disorder caused by a mutation of the Tafazzin (or TAZ) gene.

Those cells were converted into stem cells, and then grown on chips that were lined with human extracellular matrix proteins. That environment mimicked conditions in the human heart, causing the cells to join together and form into heart tissue – complete with the TAZ mutations. That tissue contracted but did so quite weakly, just as the heart tissue of a Barth syndrome patient would.

The scientists noted that the mutation caused the tissue to produce excessive amounts of reactive oxygen species (ROS), which is normally released in smaller quantities by the cells' mitochondria. By reducing the ROS production to normal levels via gene replacement therapy, however, they were able to get the diseased tissue to contract like healthy heart tissue.

They now plan on conducting animal trials, to see if a similar approach would work on a living organism. The so-called "heart disease-on-a-chip" is also finding use as a testing platform for various medications. Down the road, it's possible that patients' specific conditions could be recreated on such chips using their own cells, so that treatments could be tried outside of their body without posing any risk to the patients themselves.

A paper on the research was recently published in the journal Nature Medicine.

Source: Gizmag URL: http://www.gizmag.com/heart-disease-on-a-chip/32225/Harvard University​