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Scientists identify enzyme that trims single amino acid from mitochondrial proteins

Researchers at Heidelberg University and the University of Freiburg published a study in Nature Structural & Molecular Biology describing how an enzyme removes a specific amino acid to stabilize mitochondrial protein complexes.

New mechanism for protein maturation

The study reveals that human cells use a precise trimming process to maintain protein balance within mitochondria. Most mitochondrial proteins are manufactured outside the organelle and transported inside while carrying a molecular address sequence. Specialized enzymes remove this sequence after import, allowing the proteins to assemble into stable functional complexes.

Role of enzyme ICP55

After the initial address sequence is removed, an enzyme called ICP55 cleaves off exactly one additional amino acid. This specific step determines whether new proteins can successfully assemble into stable structures within the mitochondria. Without this precise trimming action by ICP55, numerous protein complexes lose their stability according to the findings.

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