Scientists have created functioning human heart tissue
that exhibits Barth syndrome (Image: Shutterstock)
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.
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.
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.
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.
paper on the research was recently published in the journal Nature
Source: Gizmag URL: