A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease

Acid ceramidase (aCDase) is one of several enzymes responsible for ceramide degradation within mammalian cells. As such, aCDase regulates the intracellular levels of the bioactive lipid ceramide. An inherited deficiency of aCDase activity results in Farber disease (FD), also called lipogranulomatosi...

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Bibliographic Details
Main Authors: Carmen Bedia, Luz Camacho, José Luís Abad, Gemma Fabriàs, Thierry Levade
Format: Article
Language:English
Published: Elsevier 2010-12-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520313663