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H-ABC tubulinopathy exhibits a cytoskeletal defect associated with microtubule stability

Year: 2025

Type: article

Abstract: This dataset supports the study exploring the molecular mechanisms underlying hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC), a tubulinopathy caused by mutations in TUBB4A. Using the taiep rat, a spontaneous model of the disease, we performed immunostaining on the cerebellum and corpus callosum to analyze post-translational modifications (PTMs) of tubulin, including acetylation, phosphorylation, and detyrosination. In addition, the dataset includes confocal images from HeLa cells and the oligodendrocyte cell line MO3.13 treated with paclitaxel, allowing comparisons between microtubule stability and microtubule defect in the tubulinopathy H-ABC. If you use this dataset, please cite it as indicated in the “Cite as”: Jaramillo-Restrepo, C., Piazza, V., & H Hernandez, V. (2025). H-ABC tubulinopathy exhibits a cytoskeletal defect associated with microtubule stability. https://doi.org/10.5281/zenodo.17254312

Source: Zenodo (CERN European Organization for Nuclear Research)

DOI: 10.5281/zenodo.17254311

Authors: Jaramillo-Restrepo, Carla, Piazza, Valeria, H Hernandez, Víctor

Institutions: Centro de Investigaciones en Optica, Universidad de Guanajuato

Language: English

Topic: Microtubule and mitosis dynamics

Subfield: Cell Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

OpenAlex ID: W7089571123

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