50 likes | 276 Views
All Cells Are Coated with Glycans. Glycocalyx. Cytoplasm. Nucleus. Plasma Membrane. ~50 nm. Endothelial Cell Glycocalyx. Audrey M. Glauert and G.M.W. Cook. Human lymphocyte. B. van den Berg. Symbol Nomenclature for Glycans. Mucopolysaccharidoses. Prevalence: 1:25,000
E N D
All Cells Are Coated with Glycans Glycocalyx Cytoplasm Nucleus Plasma Membrane ~50 nm Endothelial Cell Glycocalyx Audrey M. Glauert and G.M.W. Cook Human lymphocyte B. van den Berg
Mucopolysaccharidoses • Prevalence: 1:25,000 • Lysosomal storage of glycosaminoglycans, organomegaly • Many clinical features, variation in clinical onset, severity and progression • Coarse facial features, short stature • Neurological complications, enlarged or diseased heart valves, recurring respiratory infections • Early mortality
6S 6S NS 2S NS 2S -Glucuronidase 6-sulfatase 6S 6S 6S 6S NS 2S NS 2S NS 2S Sulfamidase -L-iduronidase 6S 6S 6S 6S 6S NS 2S 2S NS 2S NS 2S Uronate 2-O-sulfatase N-Acetyl Transferase 6S 6S 6S 6S -N-acetyl glucosaminidase 2S NS 2S 2S NS 2S Non-Reducing End Biomarkers MPS VII Sly MPS IIID Sanfilippo MPS IIIA Sanfilippo MPS I Hurler MPS IIIC Sanfilippo MPS II Hunter MPS IIIB Sanfilippo