Gangliosides and gangliosidoses: principles of molecular and metabolic pathogenesis

K Sandhoff, K Harzer - Journal of Neuroscience, 2013 - Soc Neuroscience
Gangliosides are the main glycolipids of neuronal plasma membranes. Their surface
patterns are generated by coordinated processes, involving biosynthetic pathways of the …

GM2 gangliosidoses: clinical features, pathophysiological aspects, and current therapies

AF Leal, E Benincore-Flórez, D Solano-Galarza… - International journal of …, 2020 - mdpi.com
GM2 gangliosidoses are a group of pathologies characterized by GM2 ganglioside
accumulation into the lysosome due to mutations on the genes encoding for the β …

Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids

T Kolter, K Sandhoff - Annu. Rev. Cell Dev. Biol., 2005 - annualreviews.org
Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell
surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and …

[HTML][HTML] Sphingolipid metabolism diseases

T Kolter, K Sandhoff - Biochimica et Biophysica Acta (BBA)-Biomembranes, 2006 - Elsevier
Human diseases caused by alterations in the metabolism of sphingolipids or
glycosphingolipids are mainly disorders of the degradation of these compounds. The …

O-GlcNAcase Uses Substrate-assisted Catalysis: Kineticanalysis And Development Of Highly Selective Mechanism-Inspiredinhibitors

MS Macauley, GE Whitworth, AW Debowski… - Journal of Biological …, 2005 - ASBMB
The post-translational modification of serine and threonine residues ofnucleocytoplasmic
proteins with 2-acetamido-2-deoxy-d-glucopyranose (GlcNAc) is a reversible process …

Ganglioside biochemistry

T Kolter - International Scholarly Research Notices, 2012 - Wiley Online Library
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 …

New approaches to Tay-Sachs disease therapy

VV Solovyeva, AA Shaimardanova… - Frontiers in …, 2018 - frontiersin.org
Tay-Sachs disease belongs to the group of autosomal-recessive lysosomal storage
metabolic disorders. This disease is caused by β-hexosaminidase A (HexA) enzyme …

Structural analysis of dispersin B, a biofilm-releasing glycoside hydrolase from the periodontopathogen Actinobacillus actinomycetemcomitans

N Ramasubbu, LM Thomas, C Ragunath… - Journal of molecular …, 2005 - Elsevier
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 …

β-N-acetylhexosaminidase: what's in a name…?

K Slámová, P Bojarová, L Petrásková, V Křen - Biotechnology Advances, 2010 - Elsevier
β-N-Acetylhexosaminidases (EC 3.2. 1.52, belonging to CAZy GH families 3, 20 and 84)
have recently gained a lot of attention, not only due to their implication in human physiology …

Sphingolipids and lysosomal pathologies

H Schulze, K Sandhoff - Biochimica et Biophysica Acta (BBA)-Molecular …, 2014 - Elsevier
Endocytosed (glyco) sphingolipids are degraded, together with other membrane lipids in a
stepwise fashion by endolysosomal enzymes with the help of small lipid binding proteins …