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Neuromuscular diseases affect millions of people
worldwide.
Now, a discovery by the Montreal Clinical Institute (IRCM) opens the door
to the development of targeted therapies.
The results, published in the journal Nature Communications, are the result
of several years of research carried out by PhD student Viviane Tran under the direction of Dr.
Jean-Franois Cté, Professor of Medicine at Université de Montréal (President and Scientific Director of IRCM).
The formation of muscles is a complex process that requires the action
of special cells, i.
e.
myoblasts.
For the development and regeneration of skeletal muscle, myoblasts must align with each other, move with each other, and touch each other until their membranes join together
.
This is known as the myoblast fusion stage and is the basis for
myofiber formation.
Myoblast fusion is crucial during embryogenesis, and mutations in certain genes can lead to an extremely rare form of clinical myopathy called Carey-Finman-Ziter syndrome
.
In adults, a large number of satellite cells are responsible for muscle growth and regeneration
.
In response to activation signals, satellite cells proliferate, differentiate, and fuse to repair damaged myofibers
.
The proteins and signaling pathways that control this fusion are still being studied
.
"Until recently, myoblast fusion was the subject of basic research," says
Dr.
Cté.
"In the context of the disease, we are not interested in it; We believe that it is impossible
to treat certain diseases with this method.
However, a detailed understanding of all the factors involved in this fusion may contribute to the development of
targeted therapies.
”
In a pivotal experiment, the researchers created a mouse model in which a protein involved in fusion was expressed
in its active form in mammals.
During muscle development and regeneration, an increase in
myoblast fusion is observed.
"We also observed that when this mouse model crossed with the limb-girdle muscular dystrophy 2B mouse model, the disease phenotype
was improved," Tran said.
Thus, the new data provide direct evidence
that myoblast fusion processes can be used for regenerative purposes and improve outcomes in muscle disease.
In the long run, this study suggests that increased cell fusion can "repair" muscles with other types of muscular dystrophy, such as Duchenne muscular dystrophy (which occurs in one in every 4,000 boys) or other serious conditions such as cachexia (secondary muscle destruction due to various diseases and some forms of cancer).
The possibility of manipulating the myoblast fusion step will undoubtedly be the subject of future research, said the researchers, who collaborated
with colleagues at UdeM's Institute of Immunology and Cancer in Montreal and internationally, as well as IRCM.
Biasing the conformation of ELMO2 reveals that myoblast fusion can be exploited to improve muscle regeneration
