First mouse model with mitochondrial tRNALeu mutation developed

Studying the role of mitochondria—the specialized structures within cells responsible for energy production—in metabolic diseases has been difficult because of a lack of animal models with the necessary mitochondrial ...

Research team first to develop 3D structure of twinkle protein

Researchers from the National Institutes of Health have developed a three-dimensional structure that allows them to see how and where disease mutations on the twinkle protein can lead to mitochondrial diseases. The protein ...

Key player in cell metabolism identified

Researchers from the Genomic Instability and Cancer Laboratory at Institute for Research in Biomedicine (IRB Barcelona) have identified a key role for EXD2 in protein production in the mitochondria, the cellular organelles ...

How cells optimize the functioning of their power plants

Mitochondria, which are probably derived from distant bacterial ancestors incorporated into our cells, have their own DNA. However, we know little about how these organelles, which convert oxygen and consumed nutrients into ...