TRNT1

Ekim 29, 2026
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The TRNT1 gene provides instructions for making a protein involved in the production (synthesis) of other proteins. During protein synthesis, a molecule called transfer RNA (tRNA) helps assemble protein building blocks (amino acids) into a chain that forms the protein. Each tRNA carries a specific amino acid to the growing chain. The TRNT1 protein modifies tRNAs by adding a series of three DNA building blocks (nucleotides), called a CCA trinucleotide, to the molecule. This modification is essential for the correct amino acid to be attached to each tRNA.

While most protein synthesis occurs in the fluid surrounding the nucleus (cytoplasm), some proteins are synthesized in cell structures called mitochondria, which are the energy-producing centers in cells. Many mitochondrial proteins form groups (complexes) that carry out the reactions that produce energy. Separate tRNA molecules are used to build proteins in the cytoplasm and mitochondria. The TRNT1 protein attaches the CCA trinucleotide to both cytoplasmic and mitochondrial tRNA molecules.

Other Names

ATP(CTP):tRNA nucleotidyltransferase; CCA tRNA nucleotidyltransferase 1, mitochondrial isoform 1; CCA tRNA nucleotidyltransferase 1, mitochondrial isoform 2; CCA-adding enzyme; CCA1; CGI-47; mitochondrial CCA-adding tRNA-nucleotidyltransferase; mt CCA-adding enzyme; mt tRNA adenylyltransferase; mt tRNA CCA-diphosphorylase; mt tRNA CCA-pyrophosphorylase; MtCCA; RPEM; SIFD; tRNA CCA nucleotidyl transferase 1; tRNA nucleotidyl transferase, CCA-adding, 1; tRNA-nucleotidyltransferase 1, mitochondrial

Related Genetics Topics

Identifiers

  • NCBI Gene: 51095
  • OMIM: 612907

Source: MedlinePlus Genetics, U.S. National Library of Medicine.

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