{"title":"麻木家族蛋白在z盘的Desmin和Vimentin定位中起作用。","authors":"Baolei Wang, Shujuan Li, Yan Yang, Jinfeng Luo","doi":"10.1007/s10974-024-09687-3","DOIUrl":null,"url":null,"abstract":"<p><p>Desmin and Vimentin are major intermediate filaments at the Z-disc and play significant roles in sarcomere architecture and signaling transduction. Abnormal expression of sarcomeric Desmin and Vimentin (SDV) results in severe dysfunctions of striated muscles. In this study, it was found that paired Numb family proteins (NFPs), including Numb and its homolog Numblike, determined the range for the recruitment of SDV to the primitive Z-disc. Notably, NFPs were identified as SDV associated proteins and bound to the head, rod, and tail domains of SDV in a splicing-variant-dependent manner. Last, the construction and consolidation of the Z-disc was completed through the gradual adjustment of the position of SDV by clockwise/anticlockwise rotation of paired NFPs to 90° in the same direction. Conditional knockout of NFPs altered the arrangement and accumulated the expression level of SDV. This study further enriches the functions of NFPs in sarcomere assembly and maintenance through cooperation with SDV.</p>","PeriodicalId":16422,"journal":{"name":"Journal of Muscle Research and Cell Motility","volume":" ","pages":""},"PeriodicalIF":1.8000,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Numb family proteins play roles in Desmin and Vimentin localization at the Z-disc.\",\"authors\":\"Baolei Wang, Shujuan Li, Yan Yang, Jinfeng Luo\",\"doi\":\"10.1007/s10974-024-09687-3\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Desmin and Vimentin are major intermediate filaments at the Z-disc and play significant roles in sarcomere architecture and signaling transduction. Abnormal expression of sarcomeric Desmin and Vimentin (SDV) results in severe dysfunctions of striated muscles. In this study, it was found that paired Numb family proteins (NFPs), including Numb and its homolog Numblike, determined the range for the recruitment of SDV to the primitive Z-disc. Notably, NFPs were identified as SDV associated proteins and bound to the head, rod, and tail domains of SDV in a splicing-variant-dependent manner. Last, the construction and consolidation of the Z-disc was completed through the gradual adjustment of the position of SDV by clockwise/anticlockwise rotation of paired NFPs to 90° in the same direction. Conditional knockout of NFPs altered the arrangement and accumulated the expression level of SDV. This study further enriches the functions of NFPs in sarcomere assembly and maintenance through cooperation with SDV.</p>\",\"PeriodicalId\":16422,\"journal\":{\"name\":\"Journal of Muscle Research and Cell Motility\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":1.8000,\"publicationDate\":\"2024-12-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Muscle Research and Cell Motility\",\"FirstCategoryId\":\"99\",\"ListUrlMain\":\"https://doi.org/10.1007/s10974-024-09687-3\",\"RegionNum\":3,\"RegionCategory\":\"生物学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CELL BIOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Muscle Research and Cell Motility","FirstCategoryId":"99","ListUrlMain":"https://doi.org/10.1007/s10974-024-09687-3","RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CELL BIOLOGY","Score":null,"Total":0}
Numb family proteins play roles in Desmin and Vimentin localization at the Z-disc.
Desmin and Vimentin are major intermediate filaments at the Z-disc and play significant roles in sarcomere architecture and signaling transduction. Abnormal expression of sarcomeric Desmin and Vimentin (SDV) results in severe dysfunctions of striated muscles. In this study, it was found that paired Numb family proteins (NFPs), including Numb and its homolog Numblike, determined the range for the recruitment of SDV to the primitive Z-disc. Notably, NFPs were identified as SDV associated proteins and bound to the head, rod, and tail domains of SDV in a splicing-variant-dependent manner. Last, the construction and consolidation of the Z-disc was completed through the gradual adjustment of the position of SDV by clockwise/anticlockwise rotation of paired NFPs to 90° in the same direction. Conditional knockout of NFPs altered the arrangement and accumulated the expression level of SDV. This study further enriches the functions of NFPs in sarcomere assembly and maintenance through cooperation with SDV.
期刊介绍：
The Journal of Muscle Research and Cell Motility has as its main aim the publication of original research which bears on either the excitation and contraction of muscle, the analysis of any one of the processes involved therein, the processes underlying contractility and motility of animal and plant cells, the toxicology and pharmacology related to contractility, or the formation, dynamics and turnover of contractile structures in muscle and non-muscle cells. Studies describing the impact of pathogenic mutations in genes encoding components of contractile structures in humans or animals are welcome, provided they offer mechanistic insight into the disease process or the underlying gene function. The policy of the Journal is to encourage any form of novel practical study whatever its specialist interest, as long as it falls within this broad field. Theoretical essays are welcome provided that they are concise and suggest practical ways in which they may be tested. Manuscripts reporting new mutations in known disease genes without validation and mechanistic insight will not be considered. It is the policy of the journal that cells lines, hybridomas and DNA clones should be made available by the developers to any qualified investigator. Submission of a manuscript for publication constitutes an agreement of the authors to abide by this principle.
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