Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases
Prohibitin 1 (PHB1) and prohibitin 2 (PHB2) are proteins that are ubiquitously expressed, and are present in the nucleus, cytosol, and mitochondria. Depending on the cellular localization, PHB1 and PHB2 have distinctive functions, but more evidence suggests a critical role within mitochondria. In fa...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2019-01-01
|
| Series: | Cells |
| Subjects: | |
| Online Access: | http://www.mdpi.com/2073-4409/8/1/71 |
| _version_ | 1797723358579654656 |
|---|---|
| author | Anna Signorile Giuseppe Sgaramella Francesco Bellomo Domenico De Rasmo |
| author_facet | Anna Signorile Giuseppe Sgaramella Francesco Bellomo Domenico De Rasmo |
| author_sort | Anna Signorile |
| collection | DOAJ |
| description | Prohibitin 1 (PHB1) and prohibitin 2 (PHB2) are proteins that are ubiquitously expressed, and are present in the nucleus, cytosol, and mitochondria. Depending on the cellular localization, PHB1 and PHB2 have distinctive functions, but more evidence suggests a critical role within mitochondria. In fact, PHB proteins are highly expressed in cells that heavily depend on mitochondrial function. In mitochondria, these two proteins assemble at the inner membrane to form a supra-macromolecular structure, which works as a scaffold for proteins and lipids regulating mitochondrial metabolism, including bioenergetics, biogenesis, and dynamics in order to determine the cell fate, death, or life. PHB alterations have been found in aging and cancer, as well as neurodegenerative, cardiac, and kidney diseases, in which significant mitochondrial impairments have been observed. The molecular mechanisms by which prohibitins regulate mitochondrial function and their role in pathology are reviewed and discussed herein. |
| first_indexed | 2024-03-12T10:01:47Z |
| format | Article |
| id | doaj.art-bdbb8a7c4de2458bb623867209646b3e |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-12T10:01:47Z |
| publishDate | 2019-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-bdbb8a7c4de2458bb623867209646b3e2023-09-02T11:41:30ZengMDPI AGCells2073-44092019-01-01817110.3390/cells8010071cells8010071Prohibitins: A Critical Role in Mitochondrial Functions and Implication in DiseasesAnna Signorile0Giuseppe Sgaramella1Francesco Bellomo2Domenico De Rasmo3Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari “Aldo Moro”, 70124 Bari, ItalyWater Research Institute (IRSA), National Research Council (CNR), Viale F. De Blasio, 5, 70132 Bari, ItalyLaboratory of Nephrology, Department of Rare Diseases, Bambino Gesù Children’s Hospital, Viale di S. Paolo, 15, 00149 Rome, ItalyInstitute of Biomembrane, Bioenergetics and Molecular Biotechnology (IBIOM), National Research Council (CNR), 70126 Bari, ItalyProhibitin 1 (PHB1) and prohibitin 2 (PHB2) are proteins that are ubiquitously expressed, and are present in the nucleus, cytosol, and mitochondria. Depending on the cellular localization, PHB1 and PHB2 have distinctive functions, but more evidence suggests a critical role within mitochondria. In fact, PHB proteins are highly expressed in cells that heavily depend on mitochondrial function. In mitochondria, these two proteins assemble at the inner membrane to form a supra-macromolecular structure, which works as a scaffold for proteins and lipids regulating mitochondrial metabolism, including bioenergetics, biogenesis, and dynamics in order to determine the cell fate, death, or life. PHB alterations have been found in aging and cancer, as well as neurodegenerative, cardiac, and kidney diseases, in which significant mitochondrial impairments have been observed. The molecular mechanisms by which prohibitins regulate mitochondrial function and their role in pathology are reviewed and discussed herein.http://www.mdpi.com/2073-4409/8/1/71mitochondriaprohibitinsapoptosisoxidative phosphorylationmitochondrial dynamics |
| spellingShingle | Anna Signorile Giuseppe Sgaramella Francesco Bellomo Domenico De Rasmo Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases Cells mitochondria prohibitins apoptosis oxidative phosphorylation mitochondrial dynamics |
| title | Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases |
| title_full | Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases |
| title_fullStr | Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases |
| title_full_unstemmed | Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases |
| title_short | Prohibitins: A Critical Role in Mitochondrial Functions and Implication in Diseases |
| title_sort | prohibitins a critical role in mitochondrial functions and implication in diseases |
| topic | mitochondria prohibitins apoptosis oxidative phosphorylation mitochondrial dynamics |
| url | http://www.mdpi.com/2073-4409/8/1/71 |
| work_keys_str_mv | AT annasignorile prohibitinsacriticalroleinmitochondrialfunctionsandimplicationindiseases AT giuseppesgaramella prohibitinsacriticalroleinmitochondrialfunctionsandimplicationindiseases AT francescobellomo prohibitinsacriticalroleinmitochondrialfunctionsandimplicationindiseases AT domenicoderasmo prohibitinsacriticalroleinmitochondrialfunctionsandimplicationindiseases |