Ceramidase

Ceramidases are enzymes that catalyze the cleavage of the N- acyl group of ceramides, as well as the reverse reaction of the acylation. They are found in eukaryotes, but also in bacteria. They are classified depending on the activity area in acidic, neutral and alkaline ceramidases. In humans, the activities of two neutral, two alkaline and acid ceramidase are occupied.

Acid ceramidase is not found in bacteria and is located within the cell in the lysosomes. It is produced as a preprotein of 395 amino acids after elimination of the signal peptide is cut into two sub- units, which together form the enzyme complex. Mutations in the gene ASAH1 of acid ceramidase can lead to Farber 's syndrome. Their expression is regulated by the ACTH signaling pathway.

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