‘Heart-on-a-chip’ project to help researchers study BMD, DMD

Duchenne Parent Project Spain invests in plan to create new lab model

Written by Marisa Wexler, MS |

A human heart is superimposed on a heart-shaped image.

Duchenne Parent Project Spain is investing €247,000 (just over $290,000 USD) in a project that aims to create a new laboratory model to study how the heart is affected in people with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD).

The “heart-on-a-chip” platform will allow researchers to grow heart cells in a three-dimensional architecture that mimics the arrangement of cells in the human body, along with sensors that can detect biologically meaningful outcomes, such as fibrosis (scarring) in the heart. The lab model could then be used to study the biology of DMD and BMD and to test experimental therapies targeting heart health.

“This breakthrough will save vital time by reducing the number of animals required, enabling us to reproduce human heart tissue affected by the disease in a laboratory setting, monitor fibrosis (scar tissue) in real time, and test drug candidates,” Marisol Montolio, director of the research department at Duchenne Parent Project Spain, said in a news story from the Institute for Bioengineering of Catalonia (IBEC).

Juan Manuel Fernández, a senior researcher in the biosensors for bioengineering group at IBEC, will lead the effort. Fernández and colleagues at IBEC will collaborate with scientists at several other institutions to make the work happen. An advisory committee of DMD families will also guide the initiative to ensure that the research aligns with community priorities.

“We will study cardiac pathology in mice and patients to build a 3D cardiac model that mimics the human disease,” Fernández said. “The heart-on-a-chip platform includes sensors that track damage and fibrosis in real time, helping us to identify and test promising treatments. This platform could also be used to study other heart diseases, accelerating drug discovery.”

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Finding balance as I manage my DMD-associated cardiomyopathy

BEAT project aims to improve cardiac care

DMD and BMD are genetic disorders marked by a deficiency of dystrophin, a protein essential for preserving muscle health. People with DMD produce virtually no dystrophin protein, while people with BMD produce a small amount, much less than normal.

In both diseases, dystrophin deficiency leads to progressive damage to muscle cells over time. This affects not only the skeletal muscles responsible for moving the body, but also cardiac muscle cells in the heart. Heart disease remains a leading cause of death among people with DMD.

This initiative to improve DMD cardiac care is called the BEAT Project. Silvia Ávila, president of Duchenne Parent Project Spain, said the name has a twofold meaning: “BEAT is the pulse of our fight,” Ávila said. “It means heartbeat, but it also means to defeat. And that’s exactly what we’re going to do with the cardiomyopathy associated with Duchenne and Becker muscular dystrophies.”

In addition to the funding from Duchenne Parent Project Spain, a crowdfunding campaign is underway to help support the initiative.

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