Structure of an enzyme crucial for tRNA maturation sheds light on cause of neurodegenerative disorders — ScienceDaily

Transfer RNAs (tRNAs) are among the most common types of RNA in a cell and are indispensable for protein production in all known organisms. They have an important “translation” function: They determine how the sequence of nucleic acids, in which the genetic information is encoded, is transcribed into a sequence of amino acids from which proteins are built.

Transfer RNAs are generated from precursor tRNAs (pre-tRNAs), which are converted in several steps into the mature tRNA with a complex three-dimensional structure. In some tRNAs, this includes a step in which a certain section, known as an intron, is excised. In humans, the tRNA splicing endonuclease (TSEN) performs this task.

The enzyme RNA kinase CLP1, which binds directly to TSEN, also plays a role in ensuring the correct conversion of tRNAs. If TSEN and CLP1 are unable to interact with each other due to a genetic mutation, it…

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