The structure of a key protein implicated in
diseases affecting the heart muscle has been
uncovered, scientists say.

Researchers, including some from King's College
London, say they now understand why faulty alpha-
actinin stops muscles from working properly when
people have the heart disease cardiomyopathy.
Their conclusions could lead to better screening and potential treatment for other conditions caused by the defect.

The research took eight years. In striated muscle, in the heart as well as skeletal muscles, alpha-actinin holds the layers at regularly spaced intervals over huge distances.

After working out the structure of the protein, the researchers, from King's College London as well as institutions in Russia, Norway and Austria, looked at what happened when it was mutated.

They showed, by tinkering with the structure, that
when key parts of the protein were faulty, heart
muscle grown in the laboratory did not form the
striations needed for it to withstand contractions.

In recent months, genetic analysis has identified
several gene mutations in alpha-actinin. Understanding the structure of the protein will now enable researchers to identify which mutated genes are actually causing problems and which have little
effect.

It will have implications for other disease where alpha-actinin has a role, such as some types of muscular dystrophy.

Prof Mathias Gautel, professor of molecular cardiology from King's College London, said: "We have worked out the structure of a major protein responsible for muscle layering.

"This gave us new insights into how muscle is built
and how its movement is controlled.
"We hope that this will help geneticists make
accurate diagnoses of muscle diseases and may
ultimately lead to designing new therapies."

Prof Jeremy Pearson, associate medical director at
the British Heart Foundation, which helped fund the
research, added: "There is currently no cure or
treatment for cardiomyopathy and these findings
demonstrate the value of funding laboratory
research to answer fundamental questions that could ultimately lead to treatments for patients."









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