Failure of fat to bond with protein in cell's powerhouse tied to inherited metabolic disorder

Photo byPhoto by National Cancer Institute on Unsplash

Researchers at Johns Hopkins Medicine have discovered that biochemical interactions between fats and proteins in mitochondria are crucial for cellular energy production. Their study, published on June 5 in The EMBO Journal, highlights how disruptions in these interactions can lead to metabolic diseases.

The research focused on the mitochondrial membrane, specifically the interactions between cardiolipin, a fatty compound, and proteins involved in transporting ATP (adenosine triphosphate), the energy molecule mitochondria produces. Mitochondria are essential for energy metabolism, and alterations in their function are linked to various metabolic disorders.

Using yeast and human cell models with mutations in mitochondrial proteins—AAC in yeast and ANT1 in humans—the scientists observed that disrupting the binding between cardiolipin and these proteins weakened their structure and function. This interference impaired the proteins' ability to transport ATP across the mitochondrial membrane, which is crucial for energy production. The study’s findings suggest that the breakdown of cardiolipin-protein interactions compromises mitochondrial function, contributing to conditions like Barth syndrome, a genetic disorder that affects the heart and muscles.

The study underscores the importance of understanding protein-lipid interactions in mitochondria, as these interactions play a crucial role in regulating energy production. This new insight could lead to identifying new therapeutic targets for treating metabolic diseases by addressing the underlying disruptions in these interactions. Further research is needed to explore the full range of protein-lipid interactions and their roles in mitochondrial function.