sphingolipidoses
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inherited sphingolipid-storage diseases, sphingolipid-storage diseases, sphingolipidosis
Physiopathology
Eukaryotic life is characterized by internal membranes of various compositions, and sphingolipids are a small but important part of these membranes.
Compositional differences between cellular membranes are maintained by sorting and sphingolipids are thought to organize this process by forming ordered domains of increased thickness in the bilayer.
Sphingolipid accumulation dysregulates the sorting of endocytic membranes.