lipidosis

lipidosis
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. [lipid + G. -osis, condition]
- ceramide lactoside l. an inherited disorder associated with an accumulation of ceramide lactoside due to a deficiency of ceramide lactosidase; results in progressive brain damage with liver and spleen enlargement.
- cerebral l. SYN: cerebral sphingolipidosis.
- cerebroside l. SYN: Gaucher disease.
- glycolipid l. SYN: Fabry disease.
- sphingomyelin l. SYN: Niemann-Pick disease.
- sulfatide l. SYN: metachromatic leukodystrophy.

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lip·i·do·sis .lip-ə-'dō-səs n, pl -do·ses -.sēz a disorder of fat metabolism esp. involving the deposition of fat in an organ (as the liver or spleen) called also lipoidosis compare LIPODYSTROPHY

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n. (pl. -ses)
any disorder of lipid metabolism within the cells of the body. The brain lipidoses (see Gaucher's disease, Hunter's syndrome, Hurler's syndrome, Tay-Sachs disease) are inborn defects causing the accumulation of lipids within the brain.

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lip·i·do·sis (lip″ĭ-doґsis) pl. lipidoґses. 1. abnormal accumulations of lipids in the tissues; see also fatty change, under change. 2. a term for several of the lysosomal storage diseases in which there is an abnormal accumulation of lipids in the reticuloendothelial cells. Called also lipid storage disease.

Medical dictionary. 2011.

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