sphingolipidosis


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sphingolipidosis

 [sfing″go-lip″ĭ-do´sis]
a general designation applied to diseases characterized by abnormal storage of sphingolipids, such as gaucher's disease, niemann-pick disease, hurler's syndrome, and tay-sachs disease; all are associated with mental retardation and premature death.

sphin·go·lip·i·do·sis

(sfing'gō-lip'i-dō'sis),
Collective designation for various diseases characterized by abnormal sphingolipid metabolism (for example, gangliosidosis, Gaucher disease, Niemann-Pick disease).

sphingolipidosis

/sphin·go·lip·i·do·sis/ (-lip″ĭ-do´sis) any of various lysosomal storage diseases characterized by abnormal storage of sphingolipids.

sphingolipidosis

(sfĭng′gō-lĭp′ĭ-dō′sĭs)
n.
Any of various diseases, such as Gaucher disease or Niemann-Pick disease, caused by a deficiency of an enzyme that degrades sphingolipids.

sphin·go·lip·i·do·sis

, sphingolipodystrophy, pl. sphingolipidoses (sfinggō-lipi-dōsis, -ō-distrŏ-fē, -i-dōsēz)
Collective designation for a variety of diseases characterized by abnormal sphingolipid metabolism, e.g., gangliosidosis, Gaucher disease, Niemann-Pick disease.

sphin·go·lip·i·do·sis

, pl. sphingo lipidoses , sphingolipodystro phy (sfinggō-lipi-dōsis, -i-dōsēz, -ō-distrŏ-fē)
Collective designation for various diseases characterized by abnormal sphingolipid metabolism.

sphingolipidosis

pl. sphingolipidoses [Gr.] a general designation applied to diseases characterized by abnormal storage of sphingolipids, such as gangliosidosis, gaucher's disease, glucocerebroside, globoid cell leukodystrophy and sphingomyelinosis.
References in periodicals archive ?
Arsenic induces oxidative stress, sphingolipidosis, depletes proteins and some antioxidants in various regions of rat brain.
In contrast to the enzymes administered in ERT, the drug used for SRT can cross the blood-brain barrier and reach target cells in the brain and thus could be used in the treatment of sphingolipidosis with involvement of the central nervous system (7).
TAY-SAGHS DISEASE is the infantile form of cerebral sphingolipidosis, a progressive disorder marked by degeneration of brain parenchymal cells and the maculas.