Based on the ganglio GSL series, gangliosides are synthesized, where sialic acids are linked to the glycan structure to produce negatively charged GSLs. (springer.com)
Gangliosides, complex glycosphingolipids on the plasma membrane containing one or more sialic acid residues, are key components of microdomains. (jneurosci.org)
Axons5
We show that activation of Neu3 sialidase, also known as Neuraminidase-3, causing conversion of GD1a and GT1b to GM1 gangliosideM1 is an essential step in regeneration occurring in PNS (sensory) but not CNS (retinal) axons in adult rat. (jneurosci.org)
In PNS axons, axotomy activates Neu3 sialidase, increasing the ratio of GM1/GD1a and GM1/GT1b gangliosides immediately after injury in vitro and in vivo . (jneurosci.org)
Externally applied sialidase converted GD1a ganglioside to GM1 and rescued axon regeneration in CNS axons and in PNS axons after Neu3 sialidase blockade. (jneurosci.org)
In this paper, we examine Neu3 sialidase action on axonal gangliosides and resultant axon regeneration, and we assess whether these mechanisms help to explain the different regeneration capacities of CNS and PNS axons. (jneurosci.org)
Furthermore, VLCFAs are postulated to be components of gangliosides and cell-adhesion molecules in growing axons and radial glia, and hence contribute to migrational defects in the CNS. (medscape.com)
Protein1
In the case of G M2 gangliosidosis, an activator protein is deficient, which leads to the accumulation of ganglioside G M2 and related glycolipids in neuronal cells. (medscape.com)
Complex2
Hydrolysis of G M2 ganglioside requires a normal G M2 ganglioside-G M2 activator-Hex A complex. (medscape.com)
A study in 1979 of initiating reactions in complex lipid syntheses in rat liver peroxisomes was conducted. (medscape.com)
Studies1
While most studies use Gb 3 extracts, chemical synthesis provides a unique tool to access different tailor-made Gb 3 glycosphingolipids. (springer.com)
Deficient2
10. Deficient ganglioside synthesis restores responsiveness to leptin and melanocortin signaling in obese KKAy mice. (nih.gov)
In the case of GM2 gangliosidosis, an activator protein is deficient, which leads to the accumulation of ganglioside GM2 and related glycolipids in neuronal cells. (medscape.com)
Biosynthesis3
3. Deficiency of ganglioside biosynthesis in metastatic human melanoma cells: relevance of CMP-NeuAc:LacCer alpha 2-3 sialyltransferase (GM3 synthase). (nih.gov)
18. Ganglioside GD3 biosynthesis in normal and mutant mouse embryos. (nih.gov)
7. Ganglioside GM3 Synthase Deficiency in Mouse Models and Human Patients. (nih.gov)
Our results suggest that ganglioside GM1 deficiency could compromise the neurotrophic effects and signals downstream of GDNF by altering the assembly of lipid raft membrane microdomains. (researchsquare.com)
In B4galnt1 knockout mice, mice with ganglioside GM1 deficiency show the characteristic symptoms of PD, such as reduced dopamine levels in the striatum, loss of dopaminergic neurons in the SNpc, aggregation of α-synuclein, and motor dysfunction. (researchsquare.com)
All these results suggest that deficiency of endogenous ganglioside GM1 may be an initiator of the pathological process of PD and play an important role in it, but the specific mechanism is not clear. (researchsquare.com)
Knockout1
11. Alteration of ganglioside synthesis by GM3 synthase knockout in murine embryonic fibroblasts. (nih.gov)
Developmental2
13. Developmental changes in ganglioside composition and synthesis in embryonic rat brain. (nih.gov)
Clinical symptoms of progressive neurodegeneration and developmental delay are caused by accumulation of GM2 gangliosideM2in lysosomes, forming membranous cytoplasmic bodies (MCBs) in neuronal cells. (medscape.com)
Genes2
Normal products of all 3 genes are required for normal catabolism of the GM2 gangliosideM2substrate. (medscape.com)
The expression of the ganglioside synthesis-related genes B3galt4 and St3gal2 is conspicuously absent in PD patients, and the GM1 level is decreased in the substantia nigra, prefrontal cortex and various peripheral tissues [ 5 , 6 ] . (researchsquare.com)
Inhibition2
Synthesis inhibition is the basis for the treatment of type 1 Gaucher disease by the glucosylceramide synthase (GCS) inhibitor eliglustat tartrate. (nih.gov)
Antibody A2B5 cyto- toxicity against retina cells is inhibited by a GQ ganglioside fraction from bovine brain (estimated half-maximal inhibition at 0.2 4M) or by N-acetylneuraminic acid (half-maximal inhi- bition at 5000 4M) but not by other purified gangliosides tested. (nih.gov)
Mice1
12. Enhanced insulin sensitivity in mice lacking ganglioside GM3. (nih.gov)
Antibodies2
Synthesis of anti-retina antibodies by hybridoma A2B5 cells was measured by com- plement-dependent cytoxicity. (nih.gov)
The common ribosome-inactivating proteins gelonin, saporin and ricin have been investigated when coupled to antibodies against neuroblastoma-enriched receptors, e.g. gangliosides GD2 and GD3 [ 6 - 8 ]. (oncotarget.com)
Signals1
Dietary ganglioside inhibits acute inflammatory signals in intestinal mucosa and blood induced by systemic inflammation of E. coli lipopolysaccharide. (davidmalab.com)
Results suggest1
These results suggest that the antigen is a complex ganglioside in plasma membranes of retina neuron cell bodies but not axons or dendrites. (nih.gov)
Plasma1
16. Trans-activity of plasma membrane-associated ganglioside sialyltransferase in mammalian cells. (nih.gov)
Cells1
19. Clostridium botulinum type C hemagglutinin affects the morphology and viability of cultured mammalian cells via binding to the ganglioside GM3. (nih.gov)