Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i>
The neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms...
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MDPI AG
2019-02-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/8/2/115 |
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| author | Meagan D. McLaren Sabateeshan Mathavarajah Robert J. Huber |
| author_facet | Meagan D. McLaren Sabateeshan Mathavarajah Robert J. Huber |
| author_sort | Meagan D. McLaren |
| collection | DOAJ |
| description | The neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms underlying the disease are unknown, in large part due to our poor understanding of the functions of NCL proteins. The social amoeba <i>Dictyostelium discoideum</i> has proven to be an exceptional model organism for studying a wide range of neurological disorders, including the NCLs. The <i>Dictyostelium</i> genome contains homologs of 11 of the 13 NCL genes. Its life cycle, comprised of both single-cell and multicellular phases, provides an excellent system for studying the effects of NCL gene deficiency on conserved cellular and developmental processes. In this review, we highlight recent advances in NCL research using <i>Dictyostelium</i> as a biomedical model. |
| first_indexed | 2024-03-12T10:43:45Z |
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| id | doaj.art-9804b82e5cff430699f77b8c71ab9b9f |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-12T10:43:45Z |
| publishDate | 2019-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-9804b82e5cff430699f77b8c71ab9b9f2023-09-02T07:54:40ZengMDPI AGCells2073-44092019-02-018211510.3390/cells8020115cells8020115Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i>Meagan D. McLaren0Sabateeshan Mathavarajah1Robert J. Huber2Department of Biology, Trent University, 1600 West Bank Drive, Peterborough, ON K9L 0G2, CanadaDepartment of Biology, Trent University, 1600 West Bank Drive, Peterborough, ON K9L 0G2, CanadaDepartment of Biology, Trent University, 1600 West Bank Drive, Peterborough, ON K9L 0G2, CanadaThe neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms underlying the disease are unknown, in large part due to our poor understanding of the functions of NCL proteins. The social amoeba <i>Dictyostelium discoideum</i> has proven to be an exceptional model organism for studying a wide range of neurological disorders, including the NCLs. The <i>Dictyostelium</i> genome contains homologs of 11 of the 13 NCL genes. Its life cycle, comprised of both single-cell and multicellular phases, provides an excellent system for studying the effects of NCL gene deficiency on conserved cellular and developmental processes. In this review, we highlight recent advances in NCL research using <i>Dictyostelium</i> as a biomedical model.https://www.mdpi.com/2073-4409/8/2/115Batten diseaseneuronal ceroid lipofuscinosis<i>Dictyostelium discoideum</i>TPP1/CLN2CLN3CLN5development |
| spellingShingle | Meagan D. McLaren Sabateeshan Mathavarajah Robert J. Huber Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> Cells Batten disease neuronal ceroid lipofuscinosis <i>Dictyostelium discoideum</i> TPP1/CLN2 CLN3 CLN5 development |
| title | Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> |
| title_full | Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> |
| title_fullStr | Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> |
| title_full_unstemmed | Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> |
| title_short | Recent Insights into NCL Protein Function Using the Model Organism <i>Dictyostelium discoideum</i> |
| title_sort | recent insights into ncl protein function using the model organism i dictyostelium discoideum i |
| topic | Batten disease neuronal ceroid lipofuscinosis <i>Dictyostelium discoideum</i> TPP1/CLN2 CLN3 CLN5 development |
| url | https://www.mdpi.com/2073-4409/8/2/115 |
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