The Role of Autophagy and Mitophagy in Huntington’s Disease
Huntington’s disease (HD) is an inherited autosomal-dominant neurodegenerative disorder that occurs due to mutations in the polyglutamine expansions of the Huntingtin protein (Htt). HD is characterised by the loss of cognitive and motor functions, as well as the development of emotional and psychiat...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Academy of Sciences Malaysia
2021-12-01
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| Series: | ASM Science Journal |
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| Online Access: | https://doi.org/10.32802/asmscj.2021.911 |
| _version_ | 1828010158953857024 |
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| author | Akhila Eswaran Crystale Siew Ying Lim Soi Moi Chye Anna Pick Kiong Ling Rhun Yian Koh |
| author_facet | Akhila Eswaran Crystale Siew Ying Lim Soi Moi Chye Anna Pick Kiong Ling Rhun Yian Koh |
| author_sort | Akhila Eswaran |
| collection | DOAJ |
| description | Huntington’s disease (HD) is an inherited autosomal-dominant neurodegenerative disorder that occurs due to mutations in the polyglutamine expansions of the Huntingtin protein (Htt). HD is characterised by the loss of cognitive and motor functions, as well as the development of emotional and psychiatric disturbances. The HD pathology is manifested through the cellular changes that arise due to the toxic functions of mutant Htt (mHtt). Autophagy is a lysosomal pathway that functions to remove damaged intracellular components while mitophagy is a selective form of autophagy involving mitochondria; and PINK1/Parkin-mediated mitophagy is the most well-understood pathway. Mitochondrial dysfunction and defects in mitophagy can be linked to the pathogenesis of HD. Previous research has shown that the presence of mHtt hinders mitophagy; while PINK1/Parkin-mediated mitophagy provides neuroprotection in HD. Hence, this review discusses the roles and regulations of mitophagy, along with an overview of mitophagy in HD. |
| first_indexed | 2024-04-10T08:49:51Z |
| format | Article |
| id | doaj.art-186485b294894cda910de1730d79d7b1 |
| institution | Directory Open Access Journal |
| issn | 1823-6782 |
| language | English |
| last_indexed | 2024-04-10T08:49:51Z |
| publishDate | 2021-12-01 |
| publisher | Academy of Sciences Malaysia |
| record_format | Article |
| series | ASM Science Journal |
| spelling | doaj.art-186485b294894cda910de1730d79d7b12023-02-22T04:16:34ZengAcademy of Sciences MalaysiaASM Science Journal1823-67822021-12-011611010.32802/asmscj.2021.911911The Role of Autophagy and Mitophagy in Huntington’s DiseaseAkhila EswaranCrystale Siew Ying LimSoi Moi ChyeAnna Pick Kiong LingRhun Yian KohHuntington’s disease (HD) is an inherited autosomal-dominant neurodegenerative disorder that occurs due to mutations in the polyglutamine expansions of the Huntingtin protein (Htt). HD is characterised by the loss of cognitive and motor functions, as well as the development of emotional and psychiatric disturbances. The HD pathology is manifested through the cellular changes that arise due to the toxic functions of mutant Htt (mHtt). Autophagy is a lysosomal pathway that functions to remove damaged intracellular components while mitophagy is a selective form of autophagy involving mitochondria; and PINK1/Parkin-mediated mitophagy is the most well-understood pathway. Mitochondrial dysfunction and defects in mitophagy can be linked to the pathogenesis of HD. Previous research has shown that the presence of mHtt hinders mitophagy; while PINK1/Parkin-mediated mitophagy provides neuroprotection in HD. Hence, this review discusses the roles and regulations of mitophagy, along with an overview of mitophagy in HD.https://doi.org/10.32802/asmscj.2021.911huntington’s diseaseautophagymitophagy |
| spellingShingle | Akhila Eswaran Crystale Siew Ying Lim Soi Moi Chye Anna Pick Kiong Ling Rhun Yian Koh The Role of Autophagy and Mitophagy in Huntington’s Disease ASM Science Journal huntington’s disease autophagy mitophagy |
| title | The Role of Autophagy and Mitophagy in Huntington’s Disease |
| title_full | The Role of Autophagy and Mitophagy in Huntington’s Disease |
| title_fullStr | The Role of Autophagy and Mitophagy in Huntington’s Disease |
| title_full_unstemmed | The Role of Autophagy and Mitophagy in Huntington’s Disease |
| title_short | The Role of Autophagy and Mitophagy in Huntington’s Disease |
| title_sort | role of autophagy and mitophagy in huntington s disease |
| topic | huntington’s disease autophagy mitophagy |
| url | https://doi.org/10.32802/asmscj.2021.911 |
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