Reply to Farber, Chabra, and Kovesi.

08:00 EDT 11th October 2017 | BioPortfolio

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Name: American journal of respiratory and critical care medicine
ISSN: 1535-4970


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Medical and Biotech [MESH] Definitions

A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.

A ceramidase subtype that is active at acid pH. It plays an important role in sphingolipid degradation by catalyzing the lysosomal hydrolysis of ceramide to sphingosine and free fatty acid. Inherited deficiency of acid ceramidase activity results in FARBER LIPOGRANULOMATOSIS.

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