End-binding 3 protein alterations in an in vitro spinal muscular atrophy model

Date

2019-09-27

Author

Koyunoğlu, Dila
Bora, Gamze
Son, Çağdaş Devrim
Yurter, Hayat

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Background/aim:Spinal muscular atrophy (SMA) is a rare neurodegenerative disease which is caused by mutations in Survival of motor neuron 1(SMN1) gene. Absence of SMN protein leads to cytoskeleton defects, especially in neurons, due to dysregulations in regulatory proteins. Our previous results showed impaired microtubule stability in SMN depleted cells and also alterations in some microtubule associated proteins, including microtubule-associated protein 1B (MAP1B). MAP1B affects the activity of microtubule plus-end protein, end-binding 3 (EB3), via regulating its microtubule binding. Therefore, in this study, we investigated the expression and localization of EB3 proteins in an in vitro SMA model. Materials and methods:SMN gene expression was knocked down by siRNA in murine motor neuron line cell line, NSC34. EB3 protein level and localization was analyzed by Western blot and immunofluorescence stainings, respectively. Quantitative microscopic analysis were performed to analyze the number and area of EB3 comets on dynamic microtubule network.Results:We detected a significant downregulation in EB3 protein level in SMN knock down cells. We also found that comet numbers were significantly increased at the beginning of neurites, however, the area of one comet on dynamic microtubules was reduced.Conclusion: Our results suggested a dysregulation in EB3 protein dynamics in the absence of SMN protein. Studies are ongoing to understand the possible link between EB3, MAP1B and their effects on microtubule stability in SMA.

Subject Keywords

Spinal muscular atrophy, Microtubule, End-binding 3

URI

https://hdl.handle.net/11511/71550
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6925586/
http://tr.molbioturk.org/MolBiyoKon19/img/MolBiyoKon19_AbstractBook.pdf

Conference Name

7th International Congress of the Molecular Biology Association of Turkey, ( 27-29 September 2019)

Collections

Department of Biology, Conference / Seminar

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Citation Formats

D. Koyunoğlu, G. Bora, Ç. D. Son, and H. Yurter, “End-binding 3 protein alterations in an in vitro spinal muscular atrophy model,” İTÜ, İstanbul, Turkey, 2019, vol. 43, Accessed: 00, 2021. [Online]. Available: https://hdl.handle.net/11511/71550.