Nature sub-journal reveals how a complex molecule moves iron in the body

A new study provides new insights into how an important class of molecules is produced and moved in human cells

Scientists have known for many years that mitochondria—specialized structures within the body's cells that are essential for respiration and energy production—are involved in the assembly and movement of iron-sulfur cofactors, the most important compounds in the body one

A new study published in the journal Nature Communications finds that these cofactors move with the help of a substance called glutathione, an antioxidant that helps by transporting these essential irons Factors cross membrane barriers and help prevent certain types of cellular damage

Glutathione is particularly useful because it helps regulate metals such as iron, which is used by red blood cells to make hemoglobin, said study co-author James Cowan, distinguished professor emeritus of chemistry and biochemistry at Ohio State University.

"Iron compounds are critical to the proper functioning of cellular biochemistry, and their assembly and transport is a complex process," Cowan said

Iron-sulfur clusters are an important class of compounds that perform a variety of metabolic processes, such as aiding electron transfer during energy production, making key metabolites in cells, and helping to replicate our genetic information

"But when these clusters don't work properly, or when key proteins don't get them, bad things happen," Cowan said

If the damaged protein fails to function, it can lead to a variety of diseases, including a rare form of anemia, Friedreich's ataxia (a disorder that causes progressive nervous system damage), and a host of other metabolic and neurological disorders

So, to study how this basic mechanism works, the researchers started with a fungus called a thermophilus, identified key protein molecules, and produced large quantities of the protein to determine the structure

Using a combination of cryo-electron microscopy and computational modeling, the team was then able to create a series of structural models detailing how mitochondria export iron cofactors to different locations in the body

"By understanding how these cofactors assemble and move around in human cells, we can lay the groundwork for determining how to prevent or reduce the symptoms of certain diseases," he said

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Structures of Atm1 provide insight into [2Fe-2S] cluster export from mitochondria