Ganglioside-induced differentiation-associated protein 1 is a type of protein that in humans is encoded by the GDAP1 gene. (wikipedia.org)
This gene encodes a member of the ganglioside-induced differentiation-associated protein family, which may play a role in a signal transduction pathway during neuronal development. (wikipedia.org)
Glycosphingolipids2
Gangliosides, complex glycosphingolipids on the plasma membrane containing one or more sialic acid residues, are key components of microdomains. (jneurosci.org)
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)
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)
Mammalian1
The adult mammalian nervous system has four dominant types of gangliosides: GM1, GD1a, GD1b, and GT1b. (jneurosci.org)
Inhibit2
Pharmacological blocking of Neu3 sialidase, downregulation of Neu3 with siRNA, or depleting gangliosides all inhibit PNS regeneration. (jneurosci.org)
We found endosomal escape does not play a role for the enhancer effect of MIC while the effect of MIC on MAP30 is cholesterol dependent and that ganglioside GM1, a lipid raft marker, can competitively inhibit cytotoxicity of MAP30 enhanced by MIC. (bvsalud.org)
GT1b3
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)
A shift in expression from the structurally simple to the structurally complex gangliosides was also observed in NB-DGJ-treated embryos and yolk sacs. (ox.ac.uk)
Mechanism1
The ASD rat model was developed in this study to demonstrate the role and mechanism of ganglioside GM1 (GM1). (bvsalud.org)
Axons2
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)
Acts1
These data further demonstrate that the ganglioside GM1 acts through its oligosaccharide also in preventing the α-synuclein pathogenic aggregation in Parkinson's disease, opening a perspective window for GM1-OS as drug candidate. (bvsalud.org)
Complex1
Hydrolysis of G M2 ganglioside requires a normal G M2 ganglioside-G M2 activator-Hex A complex. (medscape.com)
Brain2
GM1 ganglioside induces phosphorylation and activation of Trk and Erk in brain. (bio-conferences.org)
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)
We are mapping and imaging the brain's and other organs lipidome (glycolipids, cerebrosides, sphingomyelin, gangliosides and cardiolipins) and proteome in health and in pathological conditions such as blast induced traumatic brain injury, as well as mapping and imaging the anatomical distribution and localization of drugs of abuse in the addicted brain. (nih.gov)
Antibodies5
Miller-Fisher syndrome is an autoimmune neuropathy characterized by ataxia, areflexia and ophthalmoplegia, and in the majority of cases the presence of high titres of anti-GQ1b ganglioside antibodies. (nih.gov)
These findings provide clear pathological evidence to support a role for anti-ganglioside antibodies in mediating nerve terminal injury and further advance the view that this site may be of importance as a target in some human neuropathies. (nih.gov)
Concanavalin A inhibits pathophysiological effects of anti-ganglioside GQ1b antibodies at the mouse neuromuscular synapse. (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)
Inhibits1
Dietary ganglioside inhibits acute inflammatory signals in intestinal mucosa and blood induced by systemic inflammation of E. coli lipopolysaccharide. (davidmalab.com)
GD1a2
In people over 60, the total amount of gangliosides in the substantia nigra of the midbrain decreases, and this reduction is most pronounced in GM1 and GD1a [ 4 ] . (researchsquare.com)
The imaging of [M-H](-) and [M-H2O-H](-) mass peaks for GD1 gangliosides showed different distribution, most likely reflecting the different spatial distribution of GD1a and GD1b species in the brain. (nih.gov)
Lipids1
2,6-Dihydroxyacetophenone (DHA) matrix has been shown to provide efficient ionization of lipids, especially gangliosides. (nih.gov)
Neurons1
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)
Pathological1
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)
Genes2
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)
Normal products of all 3 genes are required for normal catabolism of the GM2 gangliosideM2substrate. (medscape.com)
Neuronal4
This gene encodes a member of the ganglioside-induced differentiation-associated protein family, which may play a role in a signal transduction pathway during neuronal development. (wikipedia.org)
2014). Polysialic acid neural cell adhesion molecule (PSA NCAM) and gangliosides are molecules that play important roles in neuronal development and cell adhesion in the nervous system. (veterinarska-stanica-journal.hr)
Gangliosides are a type of glycolipid found mainly in the outer leaflet of cell membranes, including neuronal membranes, and are involved in cell signalling, cell adhesion, and other cellular processes (Lopez and Schnaar, 2009) while PSA NCAM refers to a polysialic acid modification of the neural cell adhesion molecule (NCAM), a glycoprotein that plays a critical role in neurogenesis and neuron migration (Gascon et al . (veterinarska-stanica-journal.hr)
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)
Developmental1
In addition, it is important to note that the specific distribution of gangliosides and PSA NCAM may be influenced by factors such as brain region, developmental stage, and species-specific characteristics. (veterinarska-stanica-journal.hr)
Signals1
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)
Ionization1
Present characterization of ganglioside standards quantifies only the fatty acid content by gas chromatography-flame ionization detection (GC-FID) analysis of the ganglioside mixture and does not parse out the percentages of the individual mono-sialo ganglioside components. (omicsonline.org)
Major2
Gobburi ALP, Zhang R, Willard B, Inman D, Anderson DJ (2015) Heterogeneous Ganglioside Standards in LC-MS/MS: Sensitive Method for Quantifying the Major Molecular Components in Mono-Sialo Ganglioside Standards. (omicsonline.org)
A commercially-available mono-sialo (GM1) ganglioside standard consists of three major components with different ceramide structures: C18:0 fatty acid/C18-sphingosine, C18:0 fatty acid/C20-sphingosine and C20:0 fatty acid/C18-sphingosine. (omicsonline.org)
Bodies1
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)
Results1
Percentages for the individual mono-sialo gangliosides in the standard differed by at most 2% (absolute difference in the percentages) in comparing the results obtained by the two methods. (omicsonline.org)