1. Hereditary abnormality of lipid metabolism that results in abnormal amounts of lipid deposition; classification is typically based on the responsible enzymatic deficiency and type of lipid involved. Such enzymatic activity takes place in the lysosomes, and the abnormal products appear as lysosomal storage diseases. Sphingolipidoses make up the largest portion of recognized lipidoses, including abnormal metabolism of gangliosides, ceramides, and cerebrosides.
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