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Structure of a human intramembrane ceramidase explains enzymatic dysfunction found in leukodystrophy

Alkaline ceramidases (ACERs) are a class of poorly understood transmembrane enzymes controlling the homeostasis of ceramides. They are implicated in human pathophysiology, including progressive leukodystrophy, colon cancer as well as acute myeloid leukemia. We report here the crystal structure of th...

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Detalles Bibliográficos
Autores principales: Vasiliauskaité-Brooks, Ieva, Healey, Robert D., Rochaix, Pascal, Saint-Paul, Julie, Sounier, Rémy, Grison, Claire, Waltrich-Augusto, Thierry, Fortier, Mathieu, Hoh, François, Saied, Essa M., Arenz, Christoph, Basu, Shibom, Leyrat, Cédric, Granier, Sébastien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6303388/
https://www.ncbi.nlm.nih.gov/pubmed/30575723
http://dx.doi.org/10.1038/s41467-018-07864-w