An artificial muscle to study Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is the commonest muscular dystrophy recognized in childhood, with roughly 20,000 new instances reported every year. It’s a progressive muscle dysfunction that leads to the lack of muscle operate, in the end main these affected to lose their independence and expertise extreme medical points. The typical life expectancy for people with DMD is round 30 years.

The reason for the illness is a mutation within the gene encoding dystrophin, a protein that cushions the affect of muscle contraction on the cell membrane. Because of the absence of dystrophin, muscle cells are susceptible to straightforward injury.

At present, there isn’t any remedy for DMD, and one of many major challenges that the analysis group faces is to develop synthetic fashions able to precisely replicating the injury present in sufferers’ muscle tissue. That is important for finding out new therapies within the laboratory.

A examine performed by the Institute of Bioengineering of Catalonia (IBEC), printed this week within the journal Biofabrication, describes the event of a 3D muscle model able to replicating the injury discovered within the muscle tissue of people affected by Duchenne muscular dystrophy.

The system, created via tissue engineering utilizing affected person cells, consists of muscle fibers that may contract when {an electrical} stimulus is utilized. This can be a essential characteristic for establishing a man-made muscle mannequin that permits preclinical research of medication for treating DMD

The work was led by Juanma Fernández Costa, a senior researcher at IBEC, with Ainoa Tejedera Villafranca, a Ph.D. scholar at IBEC, serving as the primary writer. Each of them are a part of the Biosensors for Bioengineering group, which is led by ICREA analysis professor Javier Ramón Azcón.

“The novelty of this examine lies in our try and mannequin the first reason behind the illness, which is injury to the sarcolemma, the membrane of muscle cells. It was essential for us to copy this within the laboratory, and we now have efficiently completed it. This had not been performed beforehand,” says Juanma Fernández.

“We labored for a very long time on totally different protocols till we managed to make that injury seem within the cells of sufferers, however not within the management cells of individuals with out Duchenne. It is delicate as a result of in the event you stimulate the muscle, you may as well trigger fiber breakage in wholesome cells, simply because it occurs once we play sports activities and expertise muscle soreness,” provides Ainoa Tejedera.

The purpose of the researchers was to create a mannequin that may allow them to find out whether or not medicine can reverse this cell injury. Relatively than addressing the signs, which is the main target of palliative therapies, they aimed to focus on the foundation reason behind the illness.

Though they’ve already examined some medicine utilizing this mannequin, they’re engaged on growing an enhanced mannequin referred to as an organ-on-a-chip. It’s a extra superior platform that includes sensors and a microfluidic system into the 3D muscle mannequin. It will allow extra environment friendly monitoring of cell injury and sooner testing of varied molecules or medicine.

Offered by
Institute for Bioengineering of Catalonia