Heart Protein Helps Save Skeletal Muscles

A new study has shown that a heart muscle protein can compensate for its missing skeletal muscle counterpart to give mice suffering from muscular disease an active life.

It also increased the long-term survival of the study mice.

About Myotonic Dystrophy
Detailed information on myotonic muscular dystrophy, a common form of muscular disease that affects adults and children.

The contraction machinery protein, actin, exists in different forms in the adult heart and skeletal muscles.

The heart form, ACTC, is the dominant form in skeletal muscle of the fetus.

However during development, the skeletal form, ACTA1, increases in production and by birth has taken over.

The researchers suggested that mutations to the ACTA1 gene cause a rare but serious myopathy or muscular disease. Most patients die within the first year of life and some are born almost completely paralyzed.

Lead researcher Nowak showed that mice lacking ACTA1 died within nine days after birth.

Heart Muscle Protein may Save Life
Nowak et al have shown that a heart muscle protein has the ability to replace its skeletal counterpart and ensure a long and healthy life in lab mice

The team sought to determine if ACTC could compensate for a lack of ACTA1.

They crossed Acta1 mutant mice with transgenic mice that express human ACTC at high levels in skeletal muscle cells.

Damaged Heart Muscle Repaired With Skeletal Muscle Stem Cells
Using a novel population of stem cells, researchers at Children's Hospital of Pittsburgh of UPMC have successfully repaired damaged heart muscle in an animal model.

The resulting mice didn't die at nine days. In fact, almost 93.5pct survived more than three months, and some more than two years.

Moreover the mice's locomotor performance was comparable with wild-type, as was their overall muscle strength and their endurance was actually higher-they ran faster and for longer.

The findings were published in the Journal of Cell Biology.

Source-ANI
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