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article imageModel of human heart with cardiovascular disease made

By Tim Sandle     May 17, 2014 in Science
Using organ-on-a-chip technology, researchers have developed a model of functioning human heart tissue with an inherited cardiovascular disease.
Why would scientists want to create diseased human heart tissue? This is in order to study specific diseases to look for cures. In this recent case, it was to study Barth syndrome. Barth syndrome is a rare, untreatable cardiac disorder that affects heart and skeletal muscle function.
To study the disease further, a research group created an in-vitro chip. The chip was developed from the skin cells of two Barth syndrome patients using stem cell-based technology. It is hoped that the model will serve as a platform for better understanding the disease and testing new therapeutics to treat it.
Commenting on the study, Kevin Kit Parker of the Wyss Institute at Harvard University, an expert in organs-on-chips technology, told the Harvard Gazette: "You don’t really understand the meaning of a single cell’s genetic mutation until you build a huge chunk of organ and see how it functions or doesn’t function. In the case of the cells grown out of patients with Barth syndrome, we saw much weaker contractions and irregular tissue assembly. Being able to model the disease from a single cell all the way up to heart tissue, I think that’s a big advance."
The researchers plan to see if gene-replacement therapy will prove effective in animal models of Barth syndrome, while using the new diseased-heart-on-a-chip technology to test currently approved and experimental drugs.
The development of the chip has been published in the journal Nature Medicine. The research is headed "Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies."
More about human heart, Tissue, Cardiovascular, Cardiovascular disease, Heart attack
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