Mass spectrometry imaging for glycome in the brain
Glycans are diverse structured biomolecules that play crucial roles in various biological
processes. Glycosylation, an enzymatic system through which various glycans are bound to …
processes. Glycosylation, an enzymatic system through which various glycans are bound to …
Innate immune sensing of lysosomal dysfunction drives multiple lysosomal storage disorders
Lysosomal storage disorders (LSDs), which are characterized by genetic and metabolic
lysosomal dysfunctions, constitute over 60 degenerative diseases with considerable health …
lysosomal dysfunctions, constitute over 60 degenerative diseases with considerable health …
α-Mannosidase and β-d-N-acetylhexosaminidase outside the wall: partner exoglycosidases involved in fruit ripening process
L Morales-Quintana, A Méndez-Yáñez - Plant Molecular Biology, 2023 - Springer
Cell wall is a strong and complex net whose function is to provide turgor, pathogens attack
protection and to give structural support to the cell. In growing and expanding cells, the cell …
protection and to give structural support to the cell. In growing and expanding cells, the cell …
Bis (monoacylglycero) phosphate, a new lipid signature of endosome-derived extracellular vesicles
M Rabia, V Leuzy, C Soulage, A Durand, B Fourmaux… - Biochimie, 2020 - Elsevier
Abstract Bis (monoacylglycero) phosphate (BMP), also known as lysobisphosphatidic acid
(LBPA), is a phospholipid specifically enriched in the late endosome-lysosome compartment …
(LBPA), is a phospholipid specifically enriched in the late endosome-lysosome compartment …
Brain endothelial specific gene therapy improves experimental Sandhoff disease
G Dogbevia, H Grasshoff, A Othman… - Journal of Cerebral …, 2020 - journals.sagepub.com
In Tay-Sachs and Sandhoff disease, a deficiency of the lysosomal enzyme β-
hexosaminidase causes GM2 and other gangliosides to accumulate in neurons and triggers …
hexosaminidase causes GM2 and other gangliosides to accumulate in neurons and triggers …
Treating late-onset Tay Sachs disease: Brain delivery with a dual trojan horse protein
E Osher, Y Anis, R Singer-Shapiro, N Urshanski… - … Therapy Methods & …, 2024 - cell.com
Tay-Sachs (TS) disease is a neurodegenerative disease resulting from mutations in the
gene encoding the α-subunit (HEXA) of lysosomal β-hexosaminidase A (HexA). We report …
gene encoding the α-subunit (HEXA) of lysosomal β-hexosaminidase A (HexA). We report …
Substrate reduction therapy for sandhoff disease through inhibition of glucosylceramide synthase activity
Neuronopathic glycosphingolipidoses are a sub-group of lysosomal storage disorders for
which there are presently no effective therapies. Here, we evaluated the potential of …
which there are presently no effective therapies. Here, we evaluated the potential of …
Double prenylation of SNARE protein Ykt6 is required for lysosomal hydrolase trafficking
N Sakata, R Shirakawa, K Goto… - The journal of …, 2021 - academic.oup.com
Ykt6 is an evolutionarily conserved SNARE protein regulating Golgi membrane fusion and
other diverse membrane trafficking pathways. Unlike most SNARE proteins, Ykt6 lacks a …
other diverse membrane trafficking pathways. Unlike most SNARE proteins, Ykt6 lacks a …
Intracerebroventricular administration of a modified hexosaminidase ameliorates late-stage neurodegeneration in a GM2 mouse model
ME Lopez, D Wendt, R Lawrence, K Gong, H Ong… - PloS one, 2025 - journals.plos.org
The GM2 gangliosidoses, Tay-Sachs disease and Sandhoff disease, are devastating
neurodegenerative disorders caused by β-hexosaminidase A (HexA) deficiency. In the …
neurodegenerative disorders caused by β-hexosaminidase A (HexA) deficiency. In the …
Lysoglycosphingolipids have the ability to induce cell death through direct PI3K inhibition
R Watanabe, D Tsuji, H Tanaka, MS Uno… - Journal of …, 2023 - Wiley Online Library
Sphingolipidoses are inherited metabolic disorders associated with glycosphingolipids
accumulation, neurodegeneration, and neuroinflammation leading to severe neurological …
accumulation, neurodegeneration, and neuroinflammation leading to severe neurological …