ANKS1A-Deficiency Aberrantly Increases the Entry of the Protein Transport Machinery into the Ependymal Ciliaopen access
- Authors
- Lee, Haeryung; Lee, Jiyeon; Shin, Miram; Park, Soo Chul
- Issue Date
- Dec-2023
- Publisher
- Korean Society for Molecular and Cellular Biology
- Keywords
- ANKS1A; cilia; ependymal cell; extracellular vesicles; protein transport
- Citation
- Molecules and Cells, v.46, no.12, pp 757 - 763
- Pages
- 7
- Journal Title
- Molecules and Cells
- Volume
- 46
- Number
- 12
- Start Page
- 757
- End Page
- 763
- URI
- https://scholarworks.sookmyung.ac.kr/handle/2020.sw.sookmyung/159868
- DOI
- 10.14348/molcells.2023.0153
- ISSN
- 1016-8478
0219-1032
- Abstract
- In this study, we examine whether a change in the protein levels for FOP in Ankyrin repeat and SAM domain-containing protein 1A (ANKS1A)-deficient ependymal cells affects the intraflagellar transport (IFT) protein transport system in the multicilia. Three distinct abnormalities are observed in the multicilia of ANKS1A-deficient ependymal cells. First, there were a greater number of IFT88-positive trains along the cilia from ANKS1A deficiency. The results are similar to each isolated cilium as well. Second, each isolated cilium contains a significant increase in the number of extracellular vesicles (ECVs) due to the lack of ANKS1A. Third, Van Gogh-like 2 (Vangl2), a ciliary membrane protein, is abundantly detected along the cilia and in the ECVs attached to them for ANKS1A-deficient cells. We also use primary ependymal culture systems to obtain the ECVs released from the multicilia. Consequently, we find that ECVs from ANKS1A-deficient cells contain more IFT machinery and Vangl2. These results indicate that ANKS1A deficiency increases the entry of the protein transport machinery into the multicilia and as a result of these abnormal protein transports, excessive ECVs form along the cilia. We conclude that ependymal cells make use of the ECV-based disposal system in order to eliminate excessively transported proteins from basal bodies.
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