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. 2023 Aug 10;24(16):12645. doi: 10.3390/ijms241612645

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© 2023 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).

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Schematic representation of the localization of sphingomyelin (SM), ceramide (Cer), and sphingosine (Sph) in the plasma membrane and lysosome. Internalization of SM from the plasma membrane through endocytic vesicles to the lysosome leads to the synthesis of ceramide by the action of acid sphingomyelinase (ASM) in healthy individuals. On the other hand, in ASMD patients, SM accumulates in lysosomes due to ASM deficiency; neutral sphingomyelinase (nSM), neutral ceramidase (nCD), and acid ceramidase (ACD).