Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies
Huntington’s disease is a genetic disease caused by expanded CAG repeats on exon 1 of the huntingtin gene located on chromosome 4. Compelling evidence implicates impaired mitochondrial energetics, altered mitochondrial biogenesis and quality control, disturbed mitochondrial trafficking, oxidative st...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2023-01-01
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| Series: | Neural Regeneration Research |
| Subjects: | |
| Online Access: | http://www.nrronline.org/article.asp?issn=1673-5374;year=2023;volume=18;issue=7;spage=1472;epage=1477;aulast=Jurcau |
| _version_ | 1797954762858037248 |
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| author | Anamaria Jurcau Carolina Maria Jurcau |
| author_facet | Anamaria Jurcau Carolina Maria Jurcau |
| author_sort | Anamaria Jurcau |
| collection | DOAJ |
| description | Huntington’s disease is a genetic disease caused by expanded CAG repeats on exon 1 of the huntingtin gene located on chromosome 4. Compelling evidence implicates impaired mitochondrial energetics, altered mitochondrial biogenesis and quality control, disturbed mitochondrial trafficking, oxidative stress and mitochondrial calcium dyshomeostasis in the pathogenesis of the disorder. Unfortunately, conventional mitochondrial-targeted molecules, such as cysteamine, creatine, coenzyme Q10, or triheptanoin, yielded negative or inconclusive results. However, future therapeutic strategies, aiming to restore mitochondrial biogenesis, improving the fission/fusion balance, and improving mitochondrial trafficking, could prove useful tools in improving the phenotype of Huntington’s disease and, used in combination with genome-editing methods, could lead to a cure for the disease. |
| first_indexed | 2024-04-10T23:23:41Z |
| format | Article |
| id | doaj.art-fb22f3f7eee34b6da9c7ef498b596020 |
| institution | Directory Open Access Journal |
| issn | 1673-5374 |
| language | English |
| last_indexed | 2024-04-10T23:23:41Z |
| publishDate | 2023-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Neural Regeneration Research |
| spelling | doaj.art-fb22f3f7eee34b6da9c7ef498b5960202023-01-12T13:29:30ZengWolters Kluwer Medknow PublicationsNeural Regeneration Research1673-53742023-01-011871472147710.4103/1673-5374.360289Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategiesAnamaria JurcauCarolina Maria JurcauHuntington’s disease is a genetic disease caused by expanded CAG repeats on exon 1 of the huntingtin gene located on chromosome 4. Compelling evidence implicates impaired mitochondrial energetics, altered mitochondrial biogenesis and quality control, disturbed mitochondrial trafficking, oxidative stress and mitochondrial calcium dyshomeostasis in the pathogenesis of the disorder. Unfortunately, conventional mitochondrial-targeted molecules, such as cysteamine, creatine, coenzyme Q10, or triheptanoin, yielded negative or inconclusive results. However, future therapeutic strategies, aiming to restore mitochondrial biogenesis, improving the fission/fusion balance, and improving mitochondrial trafficking, could prove useful tools in improving the phenotype of Huntington’s disease and, used in combination with genome-editing methods, could lead to a cure for the disease.http://www.nrronline.org/article.asp?issn=1673-5374;year=2023;volume=18;issue=7;spage=1472;epage=1477;aulast=Jurcauantioxidants; calcium homeostasis; huntington’s disease; mitochondrial biogenesis; mitochondrial fission/fusion; mitochondrial trafficking; oxidative phosphorylation; oxidative stress; ss peptides; therapeutic intervention |
| spellingShingle | Anamaria Jurcau Carolina Maria Jurcau Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies Neural Regeneration Research antioxidants; calcium homeostasis; huntington’s disease; mitochondrial biogenesis; mitochondrial fission/fusion; mitochondrial trafficking; oxidative phosphorylation; oxidative stress; ss peptides; therapeutic intervention |
| title | Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies |
| title_full | Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies |
| title_fullStr | Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies |
| title_full_unstemmed | Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies |
| title_short | Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies |
| title_sort | mitochondria in huntington s disease implications in pathogenesis and mitochondrial targeted therapeutic strategies |
| topic | antioxidants; calcium homeostasis; huntington’s disease; mitochondrial biogenesis; mitochondrial fission/fusion; mitochondrial trafficking; oxidative phosphorylation; oxidative stress; ss peptides; therapeutic intervention |
| url | http://www.nrronline.org/article.asp?issn=1673-5374;year=2023;volume=18;issue=7;spage=1472;epage=1477;aulast=Jurcau |
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