lipid storage disease

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lipid storage disease

A group of rare inherited disorders of fat metabolism in which lipids are metabolized abnormally and accumulate in tissues such as the brain and peripheral nerves.

storage disease

any metabolic disorder in which some substance (e.g. fats, proteins or carbohydrates) accumulates in certain cells in abnormal amounts; called also thesaurismosis, thesaurosis.

lipid storage disease
any disorder of cellular metabolism that results in accumulation of lipids in tissues, e.g. gangliosidosis, sphingomyelinosis, gaucher's disease, globoid cell leukodystrophy, metachromatic leukodystrophy. Called also lipidosis.
lysosomal storage disease
any inborn error of metabolism in which the deficiency of a lysosomal enzyme results in the accumulation of the substance normally degraded by that enzyme in the lysosomes of certain cells. These diseases are further classified, depending on the nature of the stored substance, as glycogen storage diseases (glycogenoses), sphingolipidoses, mucopolysaccharidoses and mucolipidoses.
References in periodicals archive ?
This molecule may be effective in the treatment of Fabry's disease, a multi-systemic, lipid storage disorder caused by a deficiency of this lysosomal enzyme.
As a by-product we expect to identify putative drug targets for the treatment of degenerative diseases and lipid storage disorders, where the stabilization of the lysosomal membranes promotes cell survival.
However, in our evaluated sample, there was a preponderance of various errors of metabolism as a cause for chronic liver diseases, including storage disorders: glycogen and lipid storage disorders, in addition to errors of aminoacids and fatty acid metabolism.