Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases
Zebrafish are increasingly becoming an important model organism for studying the pathophysiological mechanisms of human diseases and investigating how these mechanisms can be effectively targeted using compounds that may open avenues to novel treatments for patients. The zebrafish skeleton has been...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-11-01
|
| Series: | Biomolecules |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2218-273X/13/11/1621 |
| _version_ | 1797459971800039424 |
|---|---|
| author | Judith Van Wynsberghe Olivier M. Vanakker |
| author_facet | Judith Van Wynsberghe Olivier M. Vanakker |
| author_sort | Judith Van Wynsberghe |
| collection | DOAJ |
| description | Zebrafish are increasingly becoming an important model organism for studying the pathophysiological mechanisms of human diseases and investigating how these mechanisms can be effectively targeted using compounds that may open avenues to novel treatments for patients. The zebrafish skeleton has been particularly instrumental in modeling bone diseases as—contrary to other model organisms—the lower load on the skeleton of an aquatic animal enables mutants to survive to early adulthood. In this respect, the axial skeletons of zebrafish have been a good read-out for congenital spinal deformities such as scoliosis and degenerative disorders such as osteoporosis and osteoarthritis, in which aberrant mineralization in humans is reflected in the respective zebrafish models. Interestingly, there have been several reports of hereditary multisystemic diseases that do not affect the vertebral column in human patients, while the corresponding zebrafish models systematically show anomalies in mineralization and morphology of the spine as their leading or, in some cases, only phenotype. In this review, we describe such examples, highlighting the underlying mechanisms, the already-used or potential power of these models to help us understand and amend the mineralization process, and the outstanding questions on how and why this specific axial type of aberrant mineralization occurs in these disease models. |
| first_indexed | 2024-03-09T16:58:32Z |
| format | Article |
| id | doaj.art-57b518cf4f0a4e25b22fc613b4105f1b |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-09T16:58:32Z |
| publishDate | 2023-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-57b518cf4f0a4e25b22fc613b4105f1b2023-11-24T14:31:59ZengMDPI AGBiomolecules2218-273X2023-11-011311162110.3390/biom13111621Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem DiseasesJudith Van Wynsberghe0Olivier M. Vanakker1Center for Medical Genetics, Ghent University Hospital, 9000 Ghent, BelgiumCenter for Medical Genetics, Ghent University Hospital, 9000 Ghent, BelgiumZebrafish are increasingly becoming an important model organism for studying the pathophysiological mechanisms of human diseases and investigating how these mechanisms can be effectively targeted using compounds that may open avenues to novel treatments for patients. The zebrafish skeleton has been particularly instrumental in modeling bone diseases as—contrary to other model organisms—the lower load on the skeleton of an aquatic animal enables mutants to survive to early adulthood. In this respect, the axial skeletons of zebrafish have been a good read-out for congenital spinal deformities such as scoliosis and degenerative disorders such as osteoporosis and osteoarthritis, in which aberrant mineralization in humans is reflected in the respective zebrafish models. Interestingly, there have been several reports of hereditary multisystemic diseases that do not affect the vertebral column in human patients, while the corresponding zebrafish models systematically show anomalies in mineralization and morphology of the spine as their leading or, in some cases, only phenotype. In this review, we describe such examples, highlighting the underlying mechanisms, the already-used or potential power of these models to help us understand and amend the mineralization process, and the outstanding questions on how and why this specific axial type of aberrant mineralization occurs in these disease models.https://www.mdpi.com/2218-273X/13/11/1621zebrafish modelmultisystemic disorderspremature mineralizationaxial skeleton |
| spellingShingle | Judith Van Wynsberghe Olivier M. Vanakker Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases Biomolecules zebrafish model multisystemic disorders premature mineralization axial skeleton |
| title | Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases |
| title_full | Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases |
| title_fullStr | Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases |
| title_full_unstemmed | Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases |
| title_short | Significance of Premature Vertebral Mineralization in Zebrafish Models in Mechanistic and Pharmaceutical Research on Hereditary Multisystem Diseases |
| title_sort | significance of premature vertebral mineralization in zebrafish models in mechanistic and pharmaceutical research on hereditary multisystem diseases |
| topic | zebrafish model multisystemic disorders premature mineralization axial skeleton |
| url | https://www.mdpi.com/2218-273X/13/11/1621 |
| work_keys_str_mv | AT judithvanwynsberghe significanceofprematurevertebralmineralizationinzebrafishmodelsinmechanisticandpharmaceuticalresearchonhereditarymultisystemdiseases AT oliviermvanakker significanceofprematurevertebralmineralizationinzebrafishmodelsinmechanisticandpharmaceuticalresearchonhereditarymultisystemdiseases |