Gangliosides: Overview | ScienceDirect Topics (2023)

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animal products

JK Aronson MA, DPhil, MBChB, FRCP, HonFBPhS, HonFFPM eDrug side effects according to Meyler, 2016


Gangliosídeoextracted from bovine brain tissue (Cronassial, Sygen) are widely used in Western Europe and South America for various neurological diseases.

Reported side effects of gangliosides include, in addition to injection site discomfort, motor neuron disease-like disorders, rash (with or without fever and nausea), and anaphylaxis. After evaluating the reported associations between ganglioside use and Guillain-Barré syndrome [29,30] In September 1994, the Committee on Proprietary Medicinal Products (CPMP) of the European Commission recommended that the marketing authorizations for Cronassial (a mixture of gangliosides for the treatment of peripheral neuropathies) be withdrawn. At the same time, the CPMP recommended the suspension of marketing authorizations for Sygen (a monosialoganglioside known as GM-1, used to treat cerebrovascular insufficiency).

In 65 patients with ischemic stroke treated intramuscularly with Sygen (monosialoganglioside 40 mg/day) for 6 weeks in a double-blind, placebo-controlled study, there were no significant differences between groups [31]. A subsequent double-blind, sequential, multicentre, randomized, placebo-controlled study of two doses of Sygen did not provide convincing evidence of efficacy.32].


Herbert Wiegandt, emComprehensive new biochemistry, 1985

3.2.1 General distribution

Gangliosídeothey are present in most if not all mammalian tissues.197]. The highest concentration of total gangliosides is found in the gray matter of the brain.200]. While the gangliosides of the central nervous system are mainly derived from the gangliosidesGanglion-Peripheral nerve series (about 0.11 µmol Neu per g wet weight [197]) and extraneural tissue (about 0.1–0.35 μmol Neu per g wet weight [200]) contain a high proportion of gangliosides from theleite-[88.461.464.496] ÖGlobus-Series [462.463]. Gangliosides ofGanglion-However, series have also been detected at extraneural sites. If in fact the presence of ganglioside Gtet1 confers sensitivity to a cell to cholera toxin (cf.Section 6.4) then a ganglioside from theGanglion- The series should be pretty ubiquitous.

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Treatment of cervical spine and spinal cord injuries

Alfredo Quinones-Hinojosa MD, emSchmidek and Sweet: Operative Neurosurgical Techniques, 2022

Use of methylprednisolone and GM-1 ganglioside

Pharmacological neuroprotection is an ongoing area of ​​research in the field of SCI. There is currently no drug approved by the US Food and Drug Administration (FDA) to treat patients with irritable bowel syndrome. Current recommendations do not support the use of methylprednisolone or GM-1Gangliosídeo.68

The use of methylprednisolone was mainly reported in three publications by Bracken et al. are reported, collectively referred to as Studies I, II, and III of the National Acute Spinal Cord Injury Study (NASCIS).69-72Although the initial results of NASCIC II70in particular they were reported to be promising, dubious post-manipulation of the data resulted in the results being only Class III recommendations.68Furthermore, the use of steroids has been associated with a higher incidence of wound ruptures and infections, VTE phenomena, dyspnea, pneumonia, sepsis and gastrointestinal bleeding, among others. Current recommendations warn against the routine use of methylprednisolone in patients with spinal cord injury; However, there are some controversies, mainly regarding its use in incomplete injuries and in extremely healthy people, such as: B. Professional athletes who may be less prone to side effects.

Ganglioside GM-1 (monosialotetrahexosylganglioside) is a mammalian cell membrane component believed to play a role in cell repair. A small study by Geisler et al. and released in 199173provided promising results for improvement in ASIA motor skill test scores at 1-year follow-up. However, Geisler's multicenter follow-up study was published in 200174could not confirm these results and no difference was found between GM-1 ganglioside and placebo at final follow-up.74a–74d). The usefulness of GM-1 ganglioside has not been studied as extensively as methylprednisolone; However, based on these 2001 results, the current recommendation is that the routine use of ganglioside GM-1 in patients with spinal cord injury be discouraged.68

Chemical Glycobiology Part A. Synthesis, Manipulation and Applications of Glycans

Naoko Komura, ... Makoto Kiso, deMethods in enzymology, 2017


Gangliosídeo, Glycosphingolipids, which contain one or more sialic acids in the glycan chain, are involved in several important biological processes in the plasma membranes (PM) of cells. However, the behavior and functions of gangliosides are poorly understood, mainly due to the lack of fluorescent analogues that correspond to native gangliosides that can be used as chemical and physical probes. In this study, we developed fully chemical methods to synthesize fluorescent gangliosides (GM3, GM2, GM1 and GD1b) in which the glucan moieties are specifically labeled with different fluorescent dyes. Functional evaluations of the synthesized fluorescent gangliosides revealed the strong influence of the fluorescent dye on the physical properties of the gangliosides in PMs and showed that the fluorescent ganglioside analogues behave similarly to the native gangliosides.

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spinal cord injury

Jean-Louis Vincent MD, PhD, emIntensive care textbook, 2017

corticosteroids and gangliosides

Several studies have shown improved neurological recovery in animals with SCI given dexamethasone or methylprednisolone.10-13Corticosteroid treatment initially showed promise as a potential therapeutic for its putative role in reducing white matter edema and inflammation. However, current evidence suggests that the main mechanism of action is to reduce the effects of secondary injury, specifically the destructive effects of lipid peroxidation on cell membranes.3Other effects include improving blood flow to the spinal cord and increasing Na activity after injury.+/K+-ATPase and facilitates extracellular calcium ion recovery.13,14

The first NASCIS study (NASCIS I) evaluated low (100 mg) and high (1000 mg) doses of methylprednisolone given to patients with acute SCI for 10 days. Unfortunately, this study had no control group and no significant difference in outcome was found, except for a higher number of wound infections in patients in the high-dose group.15The second NASCIS study (NASCIS II) was a prospective, randomized, double-blind, multicenter study that demonstrated improved neurological outcomes at 6 weeks, 6 months, and 1 year in patients with nonpenetrating spinal cord injury who received methylprednisolone treatment for a period of 30 years mg/kg bolus.sixteenImprovements in motor and sensory scores were observed associated with administration of methylprednisolone compared with naloxone or placebo, but only when the drug was administered within 8 hours of injury. The results of this study have been criticized.17,18Some of the issues relate to difficulties with randomization, reporting methods, analysis of benefit limited to small subgroups within the larger study, and lack of replication of results by an independent group of investigators, among others. NASCIS III results suggest that patients seen within 3 hours of injury should receive a bolus dose of methylprednisolone (30 mg/kg intravenously [IV]) followed by 23 hours of treatment (5 0, 4 mg/ kg/hr i.v.). ). Patients treated for 3 to 8 hours should receive the same bolus followed by a longer (48 hour) dosing schedule. Complications within 48 hours of treatment included a significant increase in severe sepsis and pneumonia.19The latest neurosurgical guidelines for the management of spinal cord injury do not recommend methylprednisolone as a treatment option because the risks of its use have been more clearly established than the benefits.20A prospective, randomized, double-blind, single-center study found a beneficial effect on functional neurological outcomes when theGangliosídeoGM-11was administered within 72 hours of human spinal cord injury.21However, a multicenter study showed no statistically significant benefit from this drug at 26 and 52 weeks after injury.22

(Video) GM 2 gangliosidosis || Biochemistry

Gangliosides in health and disease

T. August Li, Ronald L. Schnaar eAdvances in molecular biology and translational science, 2018

4 perspectives

Gangliosídeothey are the major molecular determinants of nerve cell surfaces throughout the central and peripheral nervous system, and major ganglioside structures in the brain have been well conserved during mammalian and avian evolution.1The discovery of human CDGg demonstrates the importance of gangliosides in brain development, motor and sensory function, cognition, and axon-myelin stability. The spectrum of clinical outcomes of altered ganglioside expression likely encompasses the multiple ways in which gangliosides affect cellular function. As recognition molecules on the surface of nerve cells, certain gangliosides can couple to complementary receptors on side-by-side cells, such as MAG; while other gangliosides can interact laterally at the plasma membrane level to regulate cell signaling receptors and ion channels.2,52,67,68As deletion of genes in the mouse ganglioside biosynthetic pathway leads to accumulation of gangliosides behind the blockade, some clinical findings in human CDGg may be due to higher levels of simpler structures as well as reduced levels of key brain gangliosides. As experimental insight into the functions of gangliosides in the nervous system emerges, a better understanding of the subsequent results of specific increases and decreases in ganglioside expression in the brain will follow.

The correlation between ganglioside expression and nervous system function in human diseases is currently limited by the lack of postmortem brain tissue from individuals with different diseases.B4GALNT1jST3GAL5Mutations At this time, it is not possible to correlate specific changes in ganglioside expression in the brain with variations in clinical outcomes. Likewise, results from specific mutations in the enzymatic activities of GM2/GD2 synthase and GM3 synthase are insufficient to predict clinical outcomes. In the absence of clinical specimens that shed more light on the structure-function relationships of gangliosides, the availability of patient fibroblasts provides valuable but limited information, as the ganglioside expression profile of human fibroblasts is very simple compared to that of fibroblasts. human neurons.69Using technologies to restore fibroblast pluripotency and induce neuronal differentiation,70It is expected that additional cellular materials (if not tissue) will be within reach for future studies.

As knowledge of human CDGg advances, a better understanding of the physiological and pathological role of gangliosides will emerge. This knowledge is expected to provide clinical insights beyond these rare congenital disorders and provide information that could lead to therapeutic interventions in these and other neurological diseases and disorders.

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Gangliosides in health and disease

Katsumi Matsuzaki, ... Katsuhiko Yanagisawa, enAdvances in molecular biology and translational science, 2018

5.1 GAβ production in animal models of Alzheimer's

GAβ was discovered in a human brain with early AD pathological changes4; however, GAβ production in the brain was not confirmed experimentally until the development of mAb 4396C.5To date, using the conformation-specific immunoreactivity of mAb 4396C, GAβ production in various animal models of Alzheimer's disease has been confirmed as follows. First, GAβ production was studied in the brains of non-human primates, all of which had amyloid deposits over 25 years of age.71In particular, GAβ immunoreactivity was readily observed as a granular pattern in neurons from older monkey brains, suggesting that GAβ is generated and accumulated in specific organelles with age.5This possibility was later investigated in brains obtained from animals of the same species in different age groups and it was concluded that GAβ accumulation with age occurs exclusively in endosomes.72Second, GAβ production was examined in the brains of transgenic mice engineered to express the genes responsible for familial AD and to develop extensive amyloid deposition.73In this study, GAβ was readily detected in young mouse brains before amyloid deposition became evident, in good agreement with the detection of GAβ in human brains with early but not advanced AD.73

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Organizational cell biology

K. Mihara, H. Otera, emEncyclopedia of Cell Biology, 2016

Ganglioside-Induced Differentiation Associated Protein 1 (GDAP1)

GDAP1 is another factor associated with mitochondrial fission and is localized to the MOM via a C-terminal hydrophobic transmembrane domain that extrudes most of the N-terminal domain into the cytoplasm.Niemannand others, 2005). GDPP1 is involved in thisGangliosídeoMaturation. Mutations in GDAP1 result in peripheral Charcot-Marie-Tooth neuropathy (CMT) affecting Schwann cells, myelinating glia in the peripheral nervous system, and neurons.casseroleand others, 2011). Although it is unclear how GDAP1 is involved in mitochondrial fission, these data suggest the importance of membrane lipid compositions, such as gangliosides, in mitochondrial fission. GDAP1 mutants found in patients with CMT disease do not target mitochondria and lack mitochondrial fragmentation activity (Niemannand others, 2005). GDAP1-induced mitochondrial fragmentation is inhibited by knockdown of Drp1 or expression of the dominant negative Drp1 K38A mutant, indicating that GDAP1 is a Drp1-dependent regulator of mitochondrial fission.Niemannand others, 2005).Huberand others(2013)recently reported that GDAP1 also targets peroxisomes through the action of the peroxisomal import receptor Pex19 and mediates Drp1 and Mff-dependent peroxisomal cleavage, such as in mitochondrial cleavage. GDAP1 has two domains of the glutathione S-transferase family, and overexpression of wild-type GDAP1, but not CMT disease mutants, increases cellular glutathione levels and mitochondrial membrane potential, although the relationship to functional regulation of mitochondrial morphology remain unknown.noackand others, 2012).

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The role of gangliosides and trophic factors in brain repair

A. ASCHOFF, ... B. A. SABEL, inTrophic regulation of the basal ganglia, 1994

Biochemistry of endogenous and exogenous gangliosides

Gangliosídeoare sialo-oligosaccharides attached to a ceramide moiety87They are synthesized in the cytoplasm and transported to the plasma membrane. The lipophilic ceramide unit is inserted into the the lipid bilayer and the hydrophilic oligosaccharide group with its sialic acid residues projects into the extracellular space. Ganglioside-dependent events leading to morphological and functional recovery likely involve surface modification of the plasma membrane as a result of stable insertion of exogenous gangliosides. Studies on artificial and natural membranes have shown that the distribution of gangliosides within the membrane depends on interactions between polar groups of gangliosides and cell surface proteins.89Exogenous gangliosides are spontaneously incorporated into the phospholipid bilayer and exhibit considerable lateral mobility.77Depending on the microenvironment, e.g. for example, temperature, exogenous.,84o Endogenous GM1 (Monosyaloganglyoside)23,84can concentrate in clusters and change the composition and local stability of the membrane.54The concentration of gangliosides in nerve membranes is particularly high in synaptic structures, probably due to clustering. How ganglioside cluster formation depends on intact actin44and because cytoskeletal function is restored in neurons damaged by GM1,67,83It has been suggested that GM1 may be associated with membrane and cytoskeletal proteins. Once incorporated into the membrane, exogenous gangliosides are functionally active and exhibit behavior similar to endogenous gangliosides.26,58Its location and structural diversity (more than sixty different types of molecules are now known) make the ganglioside molecule a suitable candidate as a membrane receptor or modulator of membrane-bound receptors.32,64This could explain why exogenous gangliosides have such diverse effects after brain injury.

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HUGH J. WILLISON PhD, MBBS, enAutoantibodies (second edition), 2007



Gangliosídeoare a family of up to 50 glycosphingolipids containing sialic acid, consisting of a long-chain aliphatic amine (ceramide) linked to one to five hexoses. Representative structures are shown in FIG.Table 75.1. The presence of sialic acid molecules attached to galactose residues in the core of the hexose defines a glycosphingolipid as a ganglioside. In the human nervous system is sialic acidnorte-Acetylneuraminic acid (NeuNAc). In ganglioside nomenclature, the prefix G refers to ganglion; M, D, T and Q refer to the number of sialic acid molecules (mono,di, tri or quad); Arabic numerals and lowercase letters refer to the order of migration of gangliosides on thin layer chromatograms (TLC). In general, higher molecular weight gangliosides, which have a longer oligosaccharide core and more sialic acid residues, migrate more slowly than smaller gangliosides, which move closer to the solvent front on TLC.

TABLE 75.1. Examples of clinically relevant ganglioside structures

Namecarbohydrate sequence
GM1Gal (β1–3) GalNAc (β1–4)Gal (β1–4) Glc (β1–1) Ceramida
GD1aGal (β1–3) GalNAc (β1–4)Gal (β1–4) Glc (β1–1) Ceramida
GD1bGal (β1–3) GalNAc (β1–4)Gal (β1–4) Glc (β1–1) Ceramida
GQ1bGal (β1–3) GalNAc (β1–4)Gal (β1–4) Glc (β1–1) Ceramida

biological function

Gangliosídeothey are found along plasma membranes and intracellular membranes associated with secretory and endocytic pathways. In plasma membranes, their extracellular carbohydrate units, anchored by a ceramide tail to the outer layer of the lipid bilayer, are visible to circulating autoantibodies. In peripheral nerves, both Schwann cells and axonal membranes are richly decorated with many GSLs, including gangliosides, and here act as autoantigens. The function of gangliosides is diverse, involving the modulation of a wide variety of signaling events in plasma membranes, where they are grouped into cholesterol-rich microdomains called "lipid rafts".


ImportantGangliosídeoThey are most commonly isolated from mammalian brains and can be purchased from a number of companies. Rare gangliosides that are not commercially available must be ordered in person from relevant researchers or purified in-house. Many preparations are only partially pure; B. Commercial preparations of GT1ae GT1bmay be contaminated by significant amounts of GQ1band vice versa. This should be verified in non-discriminatory immunoassays such as ELISA or dot blot. Thin layer chromatography (TLC) overlay can be helpful in this situation.

cleaning methods

Although all chemical or enzymatic synthesis ofGangliosídeoAs possible, most clinical researchers extract and purify gangliosides from bovine brain or purchase purified gangliosides from commercial sources. Different tissues vary greatly in their ganglioside composition; the nervous system is highly enriched in many types of gangliosides and is therefore the usual source of extraction. Gangliosides are purified from bovine brain by chloroform/methanol extraction and DEAE-Sephadex chromatography. Major gangliosides in the brain are GM1, DG1a, DG1be GT1b, but there are also many sources of minor gangliosides in the brain, peripheral nerves and other tissues.

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