Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment
Autosomal recessive and autosomal dominant polycystic kidney disease (ARPKD, ADPKD) are systemic disorders with pronounced hepatorenal phenotypes. While the main underlying genetic causes of both ARPKD and ADPKD have been well-known for years, the exact molecular mechanisms resulting in the observed...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-08-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/21/17/6093 |
| _version_ | 1797555996224126976 |
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| author | Sophie Haumann Roman-Ulrich Müller Max C. Liebau |
| author_facet | Sophie Haumann Roman-Ulrich Müller Max C. Liebau |
| author_sort | Sophie Haumann |
| collection | DOAJ |
| description | Autosomal recessive and autosomal dominant polycystic kidney disease (ARPKD, ADPKD) are systemic disorders with pronounced hepatorenal phenotypes. While the main underlying genetic causes of both ARPKD and ADPKD have been well-known for years, the exact molecular mechanisms resulting in the observed clinical phenotypes in the different organs, remain incompletely understood. Recent research has identified cellular metabolic changes in PKD. These findings are of major relevance as there may be an immediate translation into clinical trials and potentially clinical practice. Here, we review important results in the field regarding metabolic changes in PKD and their modulation as a potential target of systemic treatment. |
| first_indexed | 2024-03-10T16:55:28Z |
| format | Article |
| id | doaj.art-f5e8d1017c72450eab2cffc8df25f15d |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-10T16:55:28Z |
| publishDate | 2020-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-f5e8d1017c72450eab2cffc8df25f15d2023-11-20T11:10:53ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-08-012117609310.3390/ijms21176093Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic TreatmentSophie Haumann0Roman-Ulrich Müller1Max C. Liebau2Department of Pediatrics, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, GermanyDepartment II of Internal Medicine, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, GermanyDepartment of Pediatrics, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, GermanyAutosomal recessive and autosomal dominant polycystic kidney disease (ARPKD, ADPKD) are systemic disorders with pronounced hepatorenal phenotypes. While the main underlying genetic causes of both ARPKD and ADPKD have been well-known for years, the exact molecular mechanisms resulting in the observed clinical phenotypes in the different organs, remain incompletely understood. Recent research has identified cellular metabolic changes in PKD. These findings are of major relevance as there may be an immediate translation into clinical trials and potentially clinical practice. Here, we review important results in the field regarding metabolic changes in PKD and their modulation as a potential target of systemic treatment.https://www.mdpi.com/1422-0067/21/17/6093ARPKDADPKDciliapolycystinfibrocystincellular metabolism |
| spellingShingle | Sophie Haumann Roman-Ulrich Müller Max C. Liebau Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment International Journal of Molecular Sciences ARPKD ADPKD cilia polycystin fibrocystin cellular metabolism |
| title | Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment |
| title_full | Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment |
| title_fullStr | Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment |
| title_full_unstemmed | Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment |
| title_short | Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment |
| title_sort | metabolic changes in polycystic kidney disease as a potential target for systemic treatment |
| topic | ARPKD ADPKD cilia polycystin fibrocystin cellular metabolism |
| url | https://www.mdpi.com/1422-0067/21/17/6093 |
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