Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells
Cardiolipin (CL) is a unique, tetra-acylated diphosphatidylglycerol lipid that mainly localizes in the inner mitochondria membrane (IMM) in mammalian cells and plays a central role in regulating mitochondrial architecture and functioning. A deficiency of CL biosynthesis and remodeling perturbs mitoc...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-10-01
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| Series: | Genes |
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| Online Access: | https://www.mdpi.com/2073-4425/13/10/1889 |
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| author | Zhitong Jiang Tao Shen Helen Huynh Xi Fang Zhen Han Kunfu Ouyang |
| author_facet | Zhitong Jiang Tao Shen Helen Huynh Xi Fang Zhen Han Kunfu Ouyang |
| author_sort | Zhitong Jiang |
| collection | DOAJ |
| description | Cardiolipin (CL) is a unique, tetra-acylated diphosphatidylglycerol lipid that mainly localizes in the inner mitochondria membrane (IMM) in mammalian cells and plays a central role in regulating mitochondrial architecture and functioning. A deficiency of CL biosynthesis and remodeling perturbs mitochondrial functioning and ultrastructure. Clinical and experimental studies on human patients and animal models have also provided compelling evidence that an abnormal CL content, acyl chain composition, localization, and level of oxidation may be directly linked to multiple diseases, including cardiomyopathy, neuronal dysfunction, immune cell defects, and metabolic disorders. The central role of CL in regulating the pathogenesis and progression of these diseases has attracted increasing attention in recent years. In this review, we focus on the advances in our understanding of the physiological roles of CL biosynthesis and remodeling from human patients and mouse models, and we provide an overview of the potential mechanism by which CL regulates the mitochondrial architecture and functioning. |
| first_indexed | 2024-03-09T20:10:31Z |
| format | Article |
| id | doaj.art-4c067e21b34c4f00bc188b625c4c57f9 |
| institution | Directory Open Access Journal |
| issn | 2073-4425 |
| language | English |
| last_indexed | 2024-03-09T20:10:31Z |
| publishDate | 2022-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Genes |
| spelling | doaj.art-4c067e21b34c4f00bc188b625c4c57f92023-11-24T00:16:48ZengMDPI AGGenes2073-44252022-10-011310188910.3390/genes13101889Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian CellsZhitong Jiang0Tao Shen1Helen Huynh2Xi Fang3Zhen Han4Kunfu Ouyang5Department of Cardiovascular Surgery, Peking University Shenzhen Hospital, Shenzhen 518055, ChinaDepartment of Cardiovascular Surgery, Peking University Shenzhen Hospital, Shenzhen 518055, ChinaDepartment of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, San Diego, CA 92093, USADepartment of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, San Diego, CA 92093, USADepartment of Cardiovascular Surgery, Peking University Shenzhen Hospital, Shenzhen 518055, ChinaDepartment of Cardiovascular Surgery, Peking University Shenzhen Hospital, Shenzhen 518055, ChinaCardiolipin (CL) is a unique, tetra-acylated diphosphatidylglycerol lipid that mainly localizes in the inner mitochondria membrane (IMM) in mammalian cells and plays a central role in regulating mitochondrial architecture and functioning. A deficiency of CL biosynthesis and remodeling perturbs mitochondrial functioning and ultrastructure. Clinical and experimental studies on human patients and animal models have also provided compelling evidence that an abnormal CL content, acyl chain composition, localization, and level of oxidation may be directly linked to multiple diseases, including cardiomyopathy, neuronal dysfunction, immune cell defects, and metabolic disorders. The central role of CL in regulating the pathogenesis and progression of these diseases has attracted increasing attention in recent years. In this review, we focus on the advances in our understanding of the physiological roles of CL biosynthesis and remodeling from human patients and mouse models, and we provide an overview of the potential mechanism by which CL regulates the mitochondrial architecture and functioning.https://www.mdpi.com/2073-4425/13/10/1889cardiolipinmitochondriamouse modelsmitochondrial function |
| spellingShingle | Zhitong Jiang Tao Shen Helen Huynh Xi Fang Zhen Han Kunfu Ouyang Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells Genes cardiolipin mitochondria mouse models mitochondrial function |
| title | Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells |
| title_full | Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells |
| title_fullStr | Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells |
| title_full_unstemmed | Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells |
| title_short | Cardiolipin Regulates Mitochondrial Ultrastructure and Function in Mammalian Cells |
| title_sort | cardiolipin regulates mitochondrial ultrastructure and function in mammalian cells |
| topic | cardiolipin mitochondria mouse models mitochondrial function |
| url | https://www.mdpi.com/2073-4425/13/10/1889 |
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