Lysosomal storage disorders, of which more than 40 are known, are caused by the defective
activity of lysosomal proteins, which results in the intra-lysosomal accumulation of …

Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell
surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and …

Human diseases caused by alterations in the metabolism of sphingolipids or
glycosphingolipids are mainly disorders of the degradation of these compounds. The …

The post-translational modification of serine and threonine residues ofnucleocytoplasmic
proteins with 2-acetamido-2-deoxy-d-glucopyranose (GlcNAc) is a reversible process …

Gangliosides are sialic acid‐containing glycosphingolipids. They occur especially on the
cellular surfaces of neuronal cells, where they form a complex pattern, but are also found in …

Tay-Sachs disease belongs to the group of autosomal-recessive lysosomal storage
metabolic disorders. This disease is caused by β-hexosaminidase A (HexA) enzyme …

Glycoside hydrolases are important enzymes in a number of essential biological processes.
Irreversible inhibitors of this class of enzyme have attracted interest as probes of both …

Breast-fed infants often have intestinal microbiota dominated by bifidobacteria in contrast to
formula-fed infants. We found that several bifidobacterial strains produce a lacto-N …

Tay-Sachs disease, caused by impaired β-N-acetylhexosaminidase activity, was the first
GM2 gangliosidosis to be studied and one of the most severe and earliest lysosomal …

Bacteria in a biofilm are enmeshed in a self-synthesized extracellular polysaccharide matrix
that holds the bacteria together in a mass and firmly attaches the bacterial mass to the …