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

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.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2025-10-03
DOI
https://doi.org/10.5281/zenodo.17254312
Primary Topic
Avian ecology and behavior
Type
article
Field-Weighted Citation Impact
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article

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

Zenodo (CERN European Organization for Nuclear Research)
Avian ecology and behavior
article

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

article en

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.

Zenodo (CERN European Organization for Nuclear Research)
Universidad de Guanajuato (MX), Centro de Investigaciones en Optica (MX)
Openalex Percentile: Top 39%
Avian ecology and behavior
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H-ABC tubulinopathy exhibits a cytoskeletal defect associated with microtubule stability · Zenodo (CERN European Organization for Nuclear Research) (2025) | TGRS Research Map | TGRS