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lysosomal storage diseases |
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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. How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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Existing in vivo models, such as those for stroke or lysosomal storage diseases, may also prove useful for studying the structure and dynamics of the BBB. Since inception in 1997, BioMarin has applied its proprietary enzyme technology to develop products for lysosomal storage diseases and for the treatment of serious burns. He notes that MPS VII is "relatively rare" in humans, "but taken together, lysosomal storage diseases represent a significant clinical problem," especially among certain ethnic groups such as Ashkenazi Jews. |
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