Publications by authors named "Kassiani Kytidou"
Plants contain numerous glycoconjugates that are metabolized by specific glucosyltransferases and hydrolyzed by specific glycosidases, some also catalyzing synthetic transglycosylation reactions. The documented value of plant-derived glycoconjugates to beneficially modulate metabolism is first addressed. Next, focus is given to glycosidases, the central theme of the review.
View Article and Find Full Text PDFGlycosidases mediate the fragmentation of glycoconjugates in the body, including the vital recycling of endogenous molecules. Several inherited diseases in man concern deficiencies in lysosomal glycosidases degrading glycosphingolipids. Prominent is Gaucher disease caused by an impaired lysosomal β-glucosidase (glucocerebrosidase, GBA) and resulting in pathological lysosomal storage of glucosylceramide (glucocerebroside) in tissue macrophages.
View Article and Find Full Text PDFArticle Synopsis
- A deficiency in the enzyme α-galactosidase A (α-GAL) leads to Fabry disease, where there is a buildup of toxic substances in cells due to glycosphingolipid storage issues.
- Current treatment options, like enzyme replacement therapy, face challenges due to patients developing neutralizing antibodies, reducing efficacy.
- The introduction of a modified enzyme, α-NAGAL, shows promise as it has higher activity, is not neutralized by antibodies, and effectively reduces toxic levels of globotriaosylsphingosine (Lyso-Gb3) in Fabry disease patients' serum.