Homocysteine thiolactone
From Wikipedia the free encyclopedia
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| Names | |
|---|---|
| IUPAC name (3S)-3-aminothiolan-2-one | |
| Identifiers | |
3D model (JSmol) | |
| 80591 | |
| ChEBI | |
| ChEMBL | |
| ChemSpider | |
PubChem CID | |
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| Properties | |
| C4H7NOS | |
| Molar mass | 117.17 g·mol−1 |
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa). | |
Homocysteine thiolactone (HTL) is an intramolecular thioester of homocysteine synthesized by methionyl-tRNA synthetase in an error-editing reaction that prevents translational incorporation of homocysteine into proteins. It can damage proteins through homocysteinylation of protein lysine residues.[1] HTL has been reported to form isopeptide bonds with lysine residues in substrate proteins, a post-translational modification known as N-homocysteinylation (N-hcy). This causes protein damage via a thiyl radical mechanism.
When N-hcy hits α-syn, it exacerbates α-syn aggregation, neurotoxicity, and dopaminergic neuronal degeneration. It also damages the protein DJ-1, contributing to Parkinson's disease.[2]
References
[edit]- ^ Jakubowski, Hieronim (February 2000). "Homocysteine Thiolactone: Metabolic Origin and Protein Homocysteinylation in Humans". The Journal of Nutrition. 130 (2): 377S–381S. doi:10.1093/jn/130.2.377S. PMID 10721911.
- ^ Guo, Tao; Zhou, Lingyan; Xiong, Min; Xiong, Jing; Huang, Juan; Li, Yiming; Zhang, Guoxin; Chen, Guiqin; Wang, Zhi-Hao; Xiao, Tingting; Hu, Dan; Bao, Anyu; Zhang, Zhentao (May 2024). "N-homocysteinylation of DJ-1 promotes neurodegeneration in Parkinson's disease". Aging Cell. 23 (5): e14124. doi:10.1111/acel.14124. PMC 11113254. PMID 38380563.