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1. Introduction
2. Outline
3. Human mitochondrial DNA
4. Subunit proteins of respiratory chain complexes
5. Decoding system in human mitochondria
6. Basic rule of mitochondrial decoding system
7. tRNA punctuation model
8. Structural feature of mitochondrial tRNAs
9. Mitochondrial aminoacyl-tRNA synthetases
10. Indirect pathway for glutaminyl-tRNAGln synthesis
11. Post-transcriptional modification in mt-tRNA
12. The chaplet column chromatography
13. Isolation of 22 species of mt-tRNAs
14. RNA mass spectrometry
15. Determination of modified bases in tRNA
16. Bovine mitochondrial tRNA modifications
17. Post-transcriptional modifications in mt-tRNAs
18. RNA modifications in human mt-tRNAs
19. Genes responsible for tRNA modifications
20. Taurine-containing uridines in mt-tRNAs
21. mt-tRNA species bearing the taurine modification
22. Pathogenic mutations in mt-tRNA genes
23. MELAS, a point mutation in mt-tRNALeu gene
24. MELAS and MERRF
25. Direct incorporation of taurine into τm5(s2)U
26. Methylene of τm5U comes from β-carbon of Ser
27. THF is involved in τm5U formation
28. Biogenesis of τm5U
29. Mutations in MTO1 and GTPBP3
30. AUA is deciphered by tRNAMet with f5C34 (1)
31. AUA is deciphered by tRNAMet with f5C34 (2)
32. Carbon of the formyl group comes from Met
33. Sequential biogenesis of f5C34 in mt tRNAMet
34. Lack of f5C34 in NSUN3-KO cells
35. Mitochondrial dysfunction in NSUN3-KO cells
36. In vitro methylation activity of NSUN3
37. Lack of f5C34 in ALKBH1-KO cells
38. Mitochondrial dysfunction in ALKBH1-KO cells
39. In vitro oxygenase activity of ALKBH1
40. Impairment of f5C34 by pathogenic mutations
41. Summary
42. Related papers
43. Thank you

Topics Covered

• Mitochondrial tRNA modification
• tRNA modification plays a critical role in protein synthesis
• Biogenesis and pathology of taurine modification
• Biogenesis and physiology of 5-formylcytidine
• Lack of tRNA modification is a primary cause of human mitochondrial disease

Links

Series:

• Mitochondria in Health and Disease

Categories:

• Biochemistry
• Cell Biology
• Genetics & Epigenetics

Talk Citation

Publication History

• Published on May 31, 2018

Financial Disclosures

• Prof. Tsutomu Suzuki has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.