Reference - Detail
| RRC ID | 40010 |
|---|---|
| Author | Kotani Y, Morito D, Yamazaki S, Ogino K, Kawakami K, Takashima S, Hirata H, Nagata K. |
| Title | Neuromuscular regulation in zebrafish by a large AAA+ ATPase/ubiquitin ligase, mysterin/RNF213. |
| Journal | Sci Rep |
| Abstract |
Mysterin (also known as RNF213) is a huge intracellular protein with two AAA+ ATPase modules and a RING finger ubiquitin ligase domain. Mysterin was originally isolated as a significant risk factor for the cryptogenic cerebrovascular disorder moyamoya disease, and was found to be involved in physiological angiogenesis in zebrafish. However, the function and the physiological significance of mysterin in other than blood vessels remain largely unknown, although mysterin is ubiquitously expressed in animal tissues. In this study, we performed antisense-mediated suppression of a mysterin orthologue in zebrafish larvae and revealed that mysterin-deficient larvae showed significant reduction in fast myofibrils and immature projection of primary motoneurons, leading to severe motor deficits. Fast muscle-specific restoration of mysterin expression cancelled these phenotypes, and interestingly both AAA+ ATPase and ubiquitin ligase activities of mysterin were indispensable for proper fast muscle formation, demonstrating an essential role of mysterin and its enzymatic activities in the neuromuscular regulation in zebrafish. |
| Volume | 5 |
| Pages | 16161 |
| Published | 2015-11-4 |
| DOI | 10.1038/srep16161 |
| PII | srep16161 |
| PMID | 26530008 |
| PMC | PMC4632019 |
| MeSH | Animals Animals, Genetically Modified / growth & development Animals, Genetically Modified / metabolism Humans Immunohistochemistry Larva / metabolism Microscopy, Electron Motor Activity Motor Neurons / metabolism Muscle Fibers, Skeletal / metabolism Muscle Fibers, Skeletal / pathology Neovascularization, Physiologic Oligonucleotides, Antisense / metabolism RING Finger Domains Ubiquitin-Protein Ligases / antagonists & inhibitors Ubiquitin-Protein Ligases / genetics Ubiquitin-Protein Ligases / metabolism* Zebrafish / growth & development Zebrafish / metabolism* Zebrafish Proteins / antagonists & inhibitors Zebrafish Proteins / genetics Zebrafish Proteins / metabolism* |
| IF | 3.998 |
| Times Cited | 11 |
| WOS Category | MULTIDISCIPLINARY SCIENCES |
| Altmetric score |
オルトメトリクス指標項目
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| The most frequently cited source | X(Twitter) |
| Total number of mentions | 4 |
| Altmetric score changes over past 6months | 0.0 |
| Resource | |
| Zebrafish | gSA2AzGFF598A UASRFP |