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lysosomal storage diseases

   Also found in: Wikipedia 0.01 sec.
lysosomal
pertaining to or emanating from lysosomes.

lysosomal enzymes
enzymes located in the lysosomes.
lysosomal phospholipidosis
overloading of lysosomes with phospholipids as caused by the inhibition of phospholipidases by aminoglycosides.
lysosomal storage diseases
diseases in which there is a congenital or acquired deficiency of an enzyme so that one or more specific metabolic processes are not completed. As a result there is an accumulation of metabolic products in the cellular lysosomes. The histological lesion indicates the error in function but not the cause. Most of these diseases are inherited but swainsonine poisoning is caused by plant (Swainsona, Astragalus, Trachyandra spp.) poisoning. See also ceroid lipofuscinosis, glycogenosis, glycoproteinosis, lipid storage disease, mucopolysaccharidosis.

lysosomal storage diseases
A heterogeneous group of diseases with specific lysosomal enzyme defects. Cf Inborn errors of metabolism.


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Besides Hurler syndrome, Pan said the study will have positive implications in the treatment of many other lysosomal storage diseases, which affect different parts of the body, depending on the specific enzyme deficiency.
For example, there are a number of lysosomal storage diseases for which there is therapy replacing the enzyme.
uk - The Society for MPS represents 1,200 children and adults in the UK suffering from all lysosomal storage diseases.
 
 
 
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