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Hexb enzyme deficiency leads to lysosomal abnormalities in radial glia and microglia in zebrafish brain development

Sphingolipidoses are severe, mostly infantile lysosomal storage disorders (LSDs) caused by defective glycosphingolipid degradation. Two of these sphingolipidoses, Tay Sachs and Sandhoff diseases, are caused by β‐Hexosaminidase (HEXB) enzyme deficiency, resulting in ganglioside (GM2) accumulation and...

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Detalles Bibliográficos
Autores principales: Kuil, Laura E., López Martí, Anna, Carreras Mascaro, Ana, van den Bosch, Jeroen C., van den Berg, Paul, van der Linde, Herma C., Schoonderwoerd, Kees, Ruijter, George J. G., van Ham, Tjakko J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6772114/
https://www.ncbi.nlm.nih.gov/pubmed/31140649
http://dx.doi.org/10.1002/glia.23641