Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases
Caenorhabditis elegans has a number of distinct advantages that are useful for understanding the basis for cellular and organismal dysfunction underlying age-associated diseases of protein misfolding. Although protein aggregation, a key feature of human neurodegenerative diseases, has been typically...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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The Company of Biologists
2014-01-01
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| Series: | Disease Models & Mechanisms |
| Subjects: | |
| Online Access: | http://dmm.biologists.org/content/7/1/31 |
| _version_ | 1819133323526537216 |
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| author | Carmen I. Nussbaum-Krammer Richard I. Morimoto |
| author_facet | Carmen I. Nussbaum-Krammer Richard I. Morimoto |
| author_sort | Carmen I. Nussbaum-Krammer |
| collection | DOAJ |
| description | Caenorhabditis elegans has a number of distinct advantages that are useful for understanding the basis for cellular and organismal dysfunction underlying age-associated diseases of protein misfolding. Although protein aggregation, a key feature of human neurodegenerative diseases, has been typically explored in vivo at the single-cell level using cells in culture, there is now increasing evidence that proteotoxicity has a non-cell-autonomous component and is communicated between cells and tissues in a multicellular organism. These discoveries have opened up new avenues for the use of C. elegans as an ideal animal model system to study non-cell-autonomous proteotoxicity, prion-like propagation of aggregation-prone proteins, and the organismal regulation of stress responses and proteostasis. This Review focuses on recent evidence that C. elegans has mechanisms to transmit certain classes of toxic proteins between tissues and a complex stress response that integrates and coordinates signals from single cells and tissues across the organism. These findings emphasize the potential of C. elegans to provide insights into non-cell-autonomous proteotoxic mechanisms underlying age-related protein-misfolding diseases. |
| first_indexed | 2024-12-22T09:45:28Z |
| format | Article |
| id | doaj.art-1a54dfc36923448a8f29f68a00b571bf |
| institution | Directory Open Access Journal |
| issn | 1754-8403 1754-8411 |
| language | English |
| last_indexed | 2024-12-22T09:45:28Z |
| publishDate | 2014-01-01 |
| publisher | The Company of Biologists |
| record_format | Article |
| series | Disease Models & Mechanisms |
| spelling | doaj.art-1a54dfc36923448a8f29f68a00b571bf2022-12-21T18:30:33ZengThe Company of BiologistsDisease Models & Mechanisms1754-84031754-84112014-01-0171313910.1242/dmm.013011013011Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseasesCarmen I. Nussbaum-KrammerRichard I. MorimotoCaenorhabditis elegans has a number of distinct advantages that are useful for understanding the basis for cellular and organismal dysfunction underlying age-associated diseases of protein misfolding. Although protein aggregation, a key feature of human neurodegenerative diseases, has been typically explored in vivo at the single-cell level using cells in culture, there is now increasing evidence that proteotoxicity has a non-cell-autonomous component and is communicated between cells and tissues in a multicellular organism. These discoveries have opened up new avenues for the use of C. elegans as an ideal animal model system to study non-cell-autonomous proteotoxicity, prion-like propagation of aggregation-prone proteins, and the organismal regulation of stress responses and proteostasis. This Review focuses on recent evidence that C. elegans has mechanisms to transmit certain classes of toxic proteins between tissues and a complex stress response that integrates and coordinates signals from single cells and tissues across the organism. These findings emphasize the potential of C. elegans to provide insights into non-cell-autonomous proteotoxic mechanisms underlying age-related protein-misfolding diseases.http://dmm.biologists.org/content/7/1/31Caenorhabditis elegansCell non-autonomous proteotoxicityPrion-like spreading |
| spellingShingle | Carmen I. Nussbaum-Krammer Richard I. Morimoto Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases Disease Models & Mechanisms Caenorhabditis elegans Cell non-autonomous proteotoxicity Prion-like spreading |
| title | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
| title_full | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
| title_fullStr | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
| title_full_unstemmed | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
| title_short | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
| title_sort | caenorhabditis elegans as a model system for studying non cell autonomous mechanisms in protein misfolding diseases |
| topic | Caenorhabditis elegans Cell non-autonomous proteotoxicity Prion-like spreading |
| url | http://dmm.biologists.org/content/7/1/31 |
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