Researchers Explore Potential Treatment for Mitochondrial Diseases
UConn researchers are studying a group of compounds that could protect mitochondria in ways that might prevent devastating illnesses like muscular dystrophy and ALS.
Huntington’s. Parkinson’s. Muscular dystrophy. Lou Gehrig’s. These diseases share a common cause that devastatingly robs sufferers of their energy, muscle control, and in the case of Huntington’s, their sanity. But now, a group of researchers from UConn describes how a possible therapy might work.
What all those fearsome diseases have in common is dysfunctional mitochondria. Mitochondria are the body’s tiny power plants. These minuscule, rod-shaped structures inside our cells take in oxygen and nutrients and put out ATP, the body’s fuel (ATP is to cells what gasoline is to cars.) When mitochondria don’t work so well, the dysfunction can cause strange and awful symptoms that are particularly distressing in parts of the body that require lots of energy: particularly muscles, the brain, and nerve tissue.
Mitochondrial diseases tend to worsen with age. Scientists have guessed that mitochondria age as the rest of our body does. Damage acquired over time may contribute to mitochondrial diseases, but they aren’t entirely sure what’s happening or how to stop it.
“They’re insidious diseases because they rob your cells of their energy. They’re so hard to diagnose and the symptoms can be so diverse,” says Nathan Alder, a molecular biophysicist in the Department of Molecular and Cell Biology at UConn.
Alder and other researchers from UConn, the University of Texas, and Alexandria LaunchLabs are researching a group of compounds that seem to protect and even repair damage to mitochondria. The researchers describe the compounds, called SS peptides, and one potential way they may work to heal mitochondria in an upcoming issue of the Journal of Biological Chemistry.
SS peptides are made of amino acids, the building blocks of proteins, but each SS peptide is only four amino acids long. They all have the same basic plan: two amino acids with a positive charge alternating with two aromatic amino acids (“aromatic” is a chemistry term meaning they have a ring-like structure similar to benzene).
