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. Here authors solve the Xray structure of human ACER3 and uncover a Ca2+ binding site providing an explanation for the known regulatory role of Ca2+ on ACER3 activity.

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Detalles Bibliográficos
Autores principales: Ieva Vasiliauskaité-Brooks, Robert D. Healey, Pascal Rochaix, Julie Saint-Paul, Rémy Sounier, Claire Grison, Thierry Waltrich-Augusto, Mathieu Fortier, François Hoh, Essa M. Saied, Christoph Arenz, Shibom Basu, Cédric Leyrat, Sébastien Granier
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2018
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Acceso en línea:https://doaj.org/article/9944c8e0584f421dad2c7d0bf7a0b051
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Sumario:Alkaline ceramidases (ACERs) are a class of poorly understood transmembrane enzymes controlling the homeostasis of ceramides. Here authors solve the Xray structure of human ACER3 and uncover a Ca2+ binding site providing an explanation for the known regulatory role of Ca2+ on ACER3 activity.