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Mitochondrially encoded tRNA asparagine (MT-TN)
- Target
- MT-TN
- Molecular classification
- Transfer RNA (tRNA), Non-coding RNA, Other (Mitochondrially encoded RNA)
01
Overview
Mitochondrially encoded tRNA asparagine (MT-TN) is a 73-nucleotide RNA molecule encoded by the MT-TN gene of human mitochondrial DNA[1]. MT-TN transfers the amino acid asparagine to the growing polypeptide chain at the ribosome during mitochondrial protein synthesis. Mutations in MT-TN are associated with mitochondrial diseases including isolated ophthalmoplegia and cytochrome c oxidase deficiency, reflecting its essential role in the oxidative phosphorylation machinery and cellular energy production[1][2]. Mature mitochondrial tRNAs undergo a unique maturation process involving endoribonucleases, methyltransferases, and nucleotidyltransferases, ensuring correct folding and function in the structurally degenerate environment of the mitochondria[2][3].
- Other names
- trnNMT-TNmitochondrial tRNA-Asn
02
Biological functions
Protein synthesis (as tRNA, it delivers asparagine to the growing polypeptide chain during mitochondrial translation)Mitochondrial translationRNA metabolism
03
Disease associations
Complex IV deficiency / cytochrome c oxidase deficiencyIsolated ophthalmoplegia (mitochondrial disease affecting eye muscle movement)Combined complex I and IV deficiency—associated with symptoms such as failure to thrive, renal failure, mental retardation, myopathy, encephalopathy, and hypertrophic cardiomyopathy
04
Biomarkers
5692A>G (genetic biomarker for mitochondrial disorders)5703G>A (genetic biomarker for mitochondrial disorders)5728A>G (genetic biomarker for mitochondrial disorders)
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