Neuronal gangliosides. Medical search. Frequent questions

Four patients are described, who on clinical, histological, and biochemical criteria are considered to be suffering from neuronal ceroid lipofuscinosis. (bmj.com)
The aetiology of neuronal ceroid lipofuscinosis is unknown, and it is possible that there is more than one cause. (bmj.com)
Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis. (uni-kiel.de)
Lysosomal storage diseases are generally classified by the accumulated substrate and include the sphingolipidoses, oligosaccharidoses, mucolipidoses, mucopolysaccharidoses (MPSs), lipoprotein storage disorders, lysosomal transport defects, neuronal ceroid lipofuscinoses and others. (medscape.com)
Enzyme replacement therapy (ERT) appears safe and effective for peripheral manifestations in patients with Gaucher disease types I and III, Fabry disease, mucopolysaccharidosis I (Hurler, Hurler-Scheie, and Scheie syndromes), mucopolysaccharidosis II (Hunter syndrome), mucopolysaccharidosis VI (Maroteaux-Lamy syndrome), Pompe disease, and recently Batten disease (neuronal ceroid lipofuscinoses, CLN2). (medscape.com)

We reviewed the activated mechanisms of neurotoxicity in response to misfolded beta-amyloid and α-synuclein, two major toxic proteins in AD and PD, leading to neuronal apoptosis. (sagepub.com)
The collaborators determined that bone marrow transplantation could double the lifespan of Sandhoff disease mice, not by decreasing the storage of GM2 gangliosideM2 but by inhibiting activation of macrophage-derived microglial cells leading to neuronal apoptosis. (nih.gov)

The functions of gangliosides as specific determinants suggest its important role in the growth and differentiation of tissues as well as in carcinogenesis. (wikipedia.org)
It has been found that tumor formation can induce the synthesis of a new complement of ganglioside, and very low concentrations of a specific ganglioside can induce differentiation of cultured neuronal tumor cells. (wikipedia.org)
We conclude that the pattern of neuronal differentiation in CNS micromass cultures exhibits certain similarities to that observed in vivo. (nih.gov)
GM1 ganglioside plays a role in essential neuronal processes, including differentiation, survival, and signaling. (karim-cours.fr)
To further investigate the molecular pathogenesis of LSDs, the Proia/Tifft team have generated induced pluripotent stem (iPS) cells from a patient with infantile Sandhoff disease and used the CRISPR/Cas system to correct the mutation and produce isogenic iPS lines for neuronal differentiation and differential gene expression studies. (nih.gov)

10. The presence of foetal ganglioside antigens 3'-isoLM1 and 3'6'-isoLD1 in both glioma tissue and surrounding areas from human brain. (nih.gov)
Results: Twenty-eight of 33 autoimmune encephalitis patients displayed antibodies against neuronal surface or onco-neural antigens with N-methyl-D-aspartate receptor (NMDAR), glutamic acid decarboxylase (GAD) and Hu antibodies being the most prevalent. (tubitak.gov.tr)
Antibodies directed against neuronal antigens can arise in the periphery when target proteins are expressed ectopically on tumor cells. (frontiersin.org)

Guillain-Barré syndrome, which has been linked to the production of anti-ganglioside antibodies. (wikipedia.org)

Mutations in genes coding for these enzymes leads to the accumulation of partially broken down gangliosides in lysosomes, which results in a group of diseases called gangliosidosis. (wikipedia.org)
Deficient activity of these enzymes leads to accumulation of the substrate inside neuronal lysosomes, leading to cell death. (medscape.com)
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)
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)
3 The accumulation of gangliosides has been linked to several diseases including Tay-Sachs and Sandhoff disease. (matreya.com)
A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. (nih.gov)

In this study, an in vivo model of Aβ toxicity was used to investigate the effects of this peptide and the treatment with genistein on the lipid composition (gangliosides, phospholipids and cholesterol) in the frontal cortex of rats. (nih.gov)
gangliosides and phospholipids) from the milk fat globule membrane (MFGM) during pregnancy on fetal growth. (lboro.ac.uk)

7. Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors. (nih.gov)

The generation of neuronal phenotypes in the adult brain, 1-6 months post-bone marrow transplant, shows a remarkable plasticity of adult tissues with potential clinical applications, even after lethal irradiation. (nature.com)
Plays a role in regulating axon regeneration and neuronal plasticity in the adult central nervous system. (nih.gov)

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)
Characterization of cytoskeletal and neuronal markers in micromass cultures of rat embryonic midbrain cells. (nih.gov)
We present the first comparative characterization of ganglioside composition in anaplastic ganglioglioma vs. peritumoral and healthy brain tissues by combining mass spectrometry and thin-layer chromatography. (srce.hr)

Several antibodies recognize novel gangliosides. (nih.gov)
Antibody-mediated central nervous system (CNS) autoimmunity, the hallmark of several autoimmune encephalitis (AE) syndromes that produce movement and psychiatric disorders, is initiated when antibodies recognize neuronal receptors or synaptic proteins as foreign proteins ( 1 - 3 ). (frontiersin.org)

Gangliosides 1 are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially in neuronal cells in the central nervous system. (matreya.com)
Subtypes include mutations of enzymes in the BETA-N-ACETYLHEXOSAMINIDASES system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of GANGLIOSIDES , a subclass of ACIDIC GLYCOSPHINGOLIPIDS . (nih.gov)

Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. (wikipedia.org)
A similar clinical presentation seen in many neurodegenerative disorders suggests the possibility of shared neuronal responses in different disorders. (sagepub.com)

5. Ganglioside composition in human meningiomas. (nih.gov)
8. Ganglioside content and composition in human gliomas. (nih.gov)
17. Ganglioside composition and its relation to clinical data in brain tumors. (nih.gov)
Gangliosides serve as antitumor therapy targets and aberrations in their composition strongly correlate with tumor growth and invasiveness. (srce.hr)
Anaplastic ganglioglioma ganglioside composition was highly distinguishable from both peritumoral and healthy tissue despite having five to six times lower total content. (srce.hr)

Cholera Tetanus Botulism Leprosy Obesity, where inadequate ganglioside expression in mediobasal hypothalamic neurons deregulates neuronal leptin and insulin signaling. (wikipedia.org)
Pretreatment of neurons with the ganglioside GT1b (trisialosylgangliotetraglycosylceramide), followed by removal of free GT1b from the incubation medium, prevented PKC translocation, the sustained increase of 45Ca2+ uptake in the post-glutamate period, and the delayed neuronal death. (nih.gov)
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)

9. GT1b in human metastatic brain tumors: GT1b as a brain metastasis-associated ganglioside. (nih.gov)
Functions as receptor for the sialylated gangliosides GT1b and GM1 (PubMed:18411262). (nih.gov)

Sydenham's chorea and chorea in antiphospholipid syndrome or systemic lupus erythematosus are prime examples of the latter, but autoimmune chorea and dyskinesias also occur with a number of neuronal antibodies in children and adults ( Table 1 ). (medscape.com)
Serum antiganglioside antibodies in patients with autoimmune limbic en" by AYLA ÇULHA OKTAR, ÖZLEM SELÇUK et al. (tubitak.gov.tr)
Our aim was to investigate whether ganglioside antibodies are found in autoimmune encephalitis patients and may function as a diagnostic and prognostic biomarker. (tubitak.gov.tr)
Conclusion: Serum ganglioside IgM antibodies may infrequently emerge during the clinical course of autoimmune limbic encephalitis without evident polyneuropathy. (tubitak.gov.tr)
An autoimmune response against gangliosides can lead to Guillain-Barre syndrome. (matreya.com)
MFS is an autoimmune-mediated peripheral neuropathy associated with anti-GQ1b neuronal antibodies (in up to 80% of cases). (eyewiki.org)
Antibodies against neuronal receptors and synaptic proteins are associated with autoimmune encephalitides (AE) that produce movement and psychiatric disorders. (frontiersin.org)

We propose an approach for immune-mediated disorders related to neuronal, glial, or ganglioside antibodies, based on the main movement disorder presentations and the concept of isolated versus combined presentations. (medscape.com)
Anaplastic ganglioglioma is a rare, poorly characterized, malignant neuronal-glial tumor type. (srce.hr)
We also demonstrated that the flavonoid hesperidin modulates neuronal cell death by inhibiting the overexpression of inflammatory markers like nuclear factor κB, inducible nitric oxide synthase, cyclooxygenase-2 and glial fibrillary acidic protein positive astrocytes. (elsevierpure.com)

The usual multiple reaction monitoring (MRM) liquid chromatography-tandem mass spectrometry (LC-MS/MS) of gangliosides, which monitors the dehydrated sialic acid fragment (m/z 290), cannot differentiate between the individual iso-molecular weight components C18:0 fatty acid/C20-sphingosine and C20:0 fatty acid/C18-sphingosine. (omicsonline.org)
This deuterated ganglioside is ideal for the identification of gangliosides in samples and biological systems using mass spectrometry. (matreya.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)
The cst-II gene has been found to be crucial to the synthesis of ganglioside-like lipo-oligosaccharides (LOSs) that are associated with GBS and MFS. (eyewiki.org)

interruption in synaptic connections and neuronal apop- tosis [3, 4]. (sagepub.com)

Ten out of twelve MS-identified ganglioside classes, defined by unique glycan residues, were represented by a large number and considerable abundance of individual species with different fatty acid residues (C16-C24) in ceramide portions. (srce.hr)

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)
The dominant sphingoid base residue in ganglioside ceramides was sphingosine (d18:1), followed by eicosasphingosine (d20:1). (srce.hr)
Taken together, the data suggest that brain region specific regulation of gangliosides, particularly with respect to sphingosine base length, may play a role in neuronal responses to injury. (centralparkcarriagesofficial.org)

NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of gangliosides anionic at pH 7, which distinguishes them from globosides. (wikipedia.org)
GQ1b is a ganglioside - a class of lipids consisting of one or more sialic acid residues attached to the carbohydrate moiety of a glycosphingolipid. (eyewiki.org)

Background/aim: Ganglioside antibodies are identified not only in patients with inflammatory neuropathies but also several central nervous system disorders and paraneoplastic neuropathies. (tubitak.gov.tr)
Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease, Prion disease and motor disorders such as amyotrophic lateral sclerosis, present with a similar pattern of progressive neuronal death, nervous system deteriora- tion and cognitive impairment. (sagepub.com)

Recently, gangliosides have been found to be highly important molecules in immunology. (wikipedia.org)
Gangliosides are present and concentrated on cell surfaces, with the two hydrocarbon chains of the ceramide moiety embedded in the plasma membrane and the oligosaccharides located on the extracellular surface, where they present points of recognition for extracellular molecules or surfaces of neighboring cells. (wikipedia.org)
Circulating toxin molecules associate with gangliosides at a synapse of a target neuron. (reactome.org)

4. Immunohistochemical staining for ganglioside GD1b as a diagnostic and prognostic marker for primary human brain tumors. (nih.gov)
The Aβ-infused animals showed a significant decrease in ganglioside concentration and relative reduction of GD1b and GQ1b species. (nih.gov)

Nevertheless, potential impact of ganglioside antibodies on axonal degeneration and neuronal loss in limbic encephalitis pends to be further investigated. (tubitak.gov.tr)
Removal of extracellular Ca2+ (with 1mM ethyleneglycol-bis-(beta-aminoethyl ether)-N,N,N',N'-tetraacetic acid) immediately after the termination of glutamate exposure and before the appearance of the early signs of neuronal death (post-glutamate period) dramatically reduced neuronal degeneration. (nih.gov)

12. Occurrence of ganglioside GD3 in neoplastic astrocytes. (nih.gov)

Donor-derived cells expressing neuronal proteins (neuronal phenotypes) were developed in the CNS after intravascular delivery of genetically marked adult mouse bone marrow into lethally irradiated normal adult hosts, resulting in a population of donor-derived cells in the brain with distinct characteristics from the bone marrow. (nature.com)
Neuronal response in Alzheimer's and Parkinson's disease: the effect of toxic proteins on. (sagepub.com)

The name ganglioside was first applied by the German scientist Ernst Klenk in 1942 to lipids newly isolated from ganglion cells of the brain. (wikipedia.org)
3. Correlation of ganglioside patterns of primary brain tumors with survival. (nih.gov)
11. Gangliosides associated with primary brain tumors and their expression in cell lines established from these tumors. (nih.gov)
Background: Gangliosides (GAs) are important for neuronal function and development of the brain, accumulating rapidly in the fetal brain during the last trimester of pregnancy. (lboro.ac.uk)
Here, we review infectious and non-infectious triggers for AE and discuss findings from animal models of AE related to blood-brain barrier (BBB) integrity, immune cell infiltration into the CNS, and neuronal circuit dysfunction that provide useful avenues to improve diagnosis (e.g., clinical assays and imaging techniques). (frontiersin.org)

Frontal cortex was homogenized with chloroform:methanol for lipid extraction and ganglioside, phospholipid and cholesterol levels were evaluated. (nih.gov)

The situation is similar in the growing number of neuronal autoantibody-associated diseases, where movement disorder may occur in isolation, or, more frequently, combined with other signs, ranging from gross encephalopathy with altered consciousness to more subtle findings like a neuropathy. (medscape.com)
Gangliosides are also involved in several diseases: Influenza, in which haemagglutinin of influenza virus exploits certain gangliosides to enter and infect the cells expressing them. (wikipedia.org)
Isogenic disease and control iPS lines from both diseases have been differentiated into cerebral organoids in culture and faithfully replicate neuronal storage of GM2 and GM1 gangliosideM1 respectively. (nih.gov)

Biochemical analysis will show a normal ganglioside pattern, and histological examination by light and electron microscopy will reveal characteristic changes. (bmj.com)

6. Ganglioside profiles in human gliomas: quantification by microbore high performance liquid chromatography and correlation to histomorphology and grading. (nih.gov)

Studies have found that some strains of C. jejuni and H. influenza produce lipopolysaccharides that structurally resemble human gangliosides. (eyewiki.org)

Normal products of all 3 genes are required for normal catabolism of the GM2 gangliosideM2substrate. (medscape.com)

16. Myelin gangliosides of human peripheral nervous system: an enrichment of GM1 in the motor nerve myelin isolated from cauda equina. (nih.gov)

The oligosaccharide groups on gangliosides extend well beyond the surfaces of the cell membranes, and act as distinguishing surface markers that can serve as specific determinants in cellular recognition and cell-to-cell communication. (wikipedia.org)
1. Gangliosides as diagnostic markers of human astrocytomas and primitive neuroectodermal tumors. (nih.gov)
Micromass cultures of rat embryonic midbrain cells were characterized with regard to the immunolocalization of neuronal and cytoskeletal markers. (nih.gov)
Antibodies to the following markers yielded preferential staining of neuronal cells: A2B5 (GQ ganglioside), gamma-aminobutyric acid (GABA), microtubule-associated protein 2 (MAP2), MAP5, neuron-specific enolase (NSE), neural cell adhesion molecule (N-CAM), and tau. (nih.gov)

Role of tumor-associated gangliosides in cancer progression. (matreya.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)

A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. (wikipedia.org)

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)

Antibodies to beta-tubulin, c-neu, MAP1, and neurofilament (NF-H) stained both neuronal and fibroblast-like cells. (nih.gov)

However, recent studies point to a variety of humoral and cellular immune derangements that potentially result in neuronal injury and demyelination [4,5]. (who.int)

Oligodendrocytes myelinate axons, and provide for rapid conduction of neuronal signals. (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)

Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse" (Hum. (nih.gov)

Hydrolysis of GM2 gangliosideM2requires a normal GM2 ganglioside-GM2 activator-Hex A complex. (medscape.com)
Cerebrospinal fluid anti-GM1 ganglioside antibodies and oligoclonal band were negative and the IgG index was normal. (who.int)

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)

Vesicle acidification causes a conformational change in the toxin, allowing its HC part to function as a channel through which its LC part is extruded into the neuronal cytosol. (reactome.org)
Considering the roles of gangliosides on neuronal function, findings described here can contribute to the knowledge of the potential neuroprotective mechanisms of genistein against Aβ-induced alterations in the frontal cortex of rats and provide a novel view in the multifaceted scenario associated with its beneficial effects. (nih.gov)

We suggest that the sustained activation and translocation of PKC primed by glutamate receptor stimulation may be the triggering event causing the protracted increase of neuronal Ca2+ influx. (nih.gov)
Despite the difference in core elements of deposits in each neurodegenerative disorder, the cascade of neuronal reactions such as activation of glycogen synthase kinase-3 beta, mitogen-activated protein kinases, cell cycle re-entry and oxidative stress leading to a progressive neurodegeneration are surprisingly similar. (sagepub.com)

2. Ganglioside patterns in neuroepithelial tumors of childhood. (nih.gov)

It appears that this delayed increase of Ca2+ influx may be important in causing neuronal death. (nih.gov)

This complex neuronal circuitry of the spinal cord is supported by the glia of the CNS. (nih.gov)

Anatomy13

Organisms6

Diseases9

Chemicals and Drugs28

Analytical, Diagnostic and Therapeutic Techniques and Equipment4

Phenomena and Processes10

Disciplines and Occupations1

Information Science2

Ceroid5

Leading to neuronal apoptosis2

Differentiation5

Antigens3

Anti-gangliosid1

Accumulation6

Phospholipids2

Glycolipids1

Plasticity2

Characterization3

Antibodies recognize2

Acidic glycosphingolipids2

Neurodegenerative disorders2

Composition5

Neurons3

GT1b2

Autoimmune7

Glial3

Mass spectrometry2

Synthesis2

Synaptic1

Glycan1

Sphingosine3

Sialic acid2

Disorders2

Molecules3

GD1b2

Degeneration2

Occurrence1

Proteins2

Brain5

Phospholipid1

Diseases3

Biochemical1

Quantification1

Influenza1

Genes1

Peripheral1

Markers4

Role2

Ceramide1

Midbrain1

Cells1

Cellular1

Signals2

Clinical course1

Normal2

Molecular1

Function2

Activation2

Childhood1

Important1

Complex1