Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels
Cysteine is a semi-essential amino acid that not only plays an essential role as a component of protein synthesis, but also in the generation of numerous sulfur-containing molecules such as the antioxidant glutathione and coenzyme A. We previously showed that the metabolism of cysteine is dysregulat...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-07-01
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| Series: | Antioxidants |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2076-3921/11/8/1470 |
| _version_ | 1827600981362212864 |
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| author | Bindu D. Paul Juan I. Sbodio Solomon H. Snyder |
| author_facet | Bindu D. Paul Juan I. Sbodio Solomon H. Snyder |
| author_sort | Bindu D. Paul |
| collection | DOAJ |
| description | Cysteine is a semi-essential amino acid that not only plays an essential role as a component of protein synthesis, but also in the generation of numerous sulfur-containing molecules such as the antioxidant glutathione and coenzyme A. We previously showed that the metabolism of cysteine is dysregulated in Huntington’s disease (HD), a neurodegenerative disorder triggered by the expansion of polyglutamine repeats in the protein huntingtin. In this study, we showed that cysteine metabolism is compromised at multiple levels in HD, both transcriptional and post-translational. Accordingly, restoring cysteine homeostasis may be beneficial in HD. |
| first_indexed | 2024-03-09T04:45:48Z |
| format | Article |
| id | doaj.art-429bc619186142979048b365f688669c |
| institution | Directory Open Access Journal |
| issn | 2076-3921 |
| language | English |
| last_indexed | 2024-03-09T04:45:48Z |
| publishDate | 2022-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Antioxidants |
| spelling | doaj.art-429bc619186142979048b365f688669c2023-12-03T13:15:25ZengMDPI AGAntioxidants2076-39212022-07-01118147010.3390/antiox11081470Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational LevelsBindu D. Paul0Juan I. Sbodio1Solomon H. Snyder2Department of Pharmacology and Molecular Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAThe Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pharmacology and Molecular Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USACysteine is a semi-essential amino acid that not only plays an essential role as a component of protein synthesis, but also in the generation of numerous sulfur-containing molecules such as the antioxidant glutathione and coenzyme A. We previously showed that the metabolism of cysteine is dysregulated in Huntington’s disease (HD), a neurodegenerative disorder triggered by the expansion of polyglutamine repeats in the protein huntingtin. In this study, we showed that cysteine metabolism is compromised at multiple levels in HD, both transcriptional and post-translational. Accordingly, restoring cysteine homeostasis may be beneficial in HD.https://www.mdpi.com/2076-3921/11/8/1470Huntington’s diseasecysteinecystathionine γ-lyasetranssulfuration |
| spellingShingle | Bindu D. Paul Juan I. Sbodio Solomon H. Snyder Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels Antioxidants Huntington’s disease cysteine cystathionine γ-lyase transsulfuration |
| title | Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels |
| title_full | Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels |
| title_fullStr | Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels |
| title_full_unstemmed | Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels |
| title_short | Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels |
| title_sort | mutant huntingtin derails cysteine metabolism in huntington s disease at both transcriptional and post translational levels |
| topic | Huntington’s disease cysteine cystathionine γ-lyase transsulfuration |
| url | https://www.mdpi.com/2076-3921/11/8/1470 |
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