Cholesteryl ester storage disease (CESD) is caused by deficient lysosomal acid lipase (LAL)
activity, predominantly resulting in cholesteryl ester (CE) accumulation, particularly in the …

Lysosomes and lysosomal enzymes play a central role in numerous cellular processes,
including cellular nutrition, recycling, signaling, defense, and cell death. Genetic deficiencies …

Niemann-Pick type C disease is largely attributable to an inactivating mutation of NPC1
protein, which normally aids movement of unesterified cholesterol (C) from the …

BACKGROUND: Cholesterol ester storage disease (CESD) and Wolman Disease (WD) are
due to deficiency of lysosomal acid lipase (LAL). A new method is described for the …

Plants have been proposed as an attractive alternative for pharmaceutical protein
production to current mammalian or microbial cell‐based systems. Eukaryotic protein …

Lysosomal lipid storage diseases, or lipidoses, are inherited metabolic disorders in which
typically lipids accumulate in cells and tissues. Complex lipids, such as glycosphingolipids …

While unesterified cholesterol (C) is essential for remodeling neuronal plasma membranes,
its role in certain neurodegenerative disorders remains poorly defined. Uptake of sterol from …

Gaucher disease (GD) is a rare, genetic lysosomal storage disorder caused by functional
defects of acid β-glucosidase that results in multiple organ dysfunction. Glycosylation of …

Niemann-Pick disease type C (NPC) and Wolman disease are two members of a family of
storage disorders caused by mutations of genes encoding lysosomal proteins. Deficiency in …

Hepatic steatosis is one of the most common liver disorders in the general population. The
main cause of hepatic steatosis is nonalcoholic fatty liver disease (NAFLD), representing the …