Page last updated: 2024-11-13

globotriaosylceramide

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Description

globotriaosylceramide: receptor for Shigella [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide : A glycotriaosylceramide having alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl component attached to the primary hydroxy function of a ceramide with undefined sphingoid base. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Cross-References

ID SourceID
PubMed CID66616222
CHEBI ID18313
SCHEMBL ID182663
MeSH IDM0070209

Synonyms (28)

Synonym
globotriaosylceramide
trihexosylceramide
gal-alpha1->4laccer
d-galactosyl-1,4-d-galactosyl-1,4-d-glucosylceramide
alpha-d-galactosyl-(1->4)-beta-d-galactosyl-(1->4)-beta-d-glucosyl-(1<->1)-ceramide
globotriosylceramide
gal-alpha1->4gal-beta1->4glc-beta1->1'cer
CHEBI:18313 ,
alpha-d-galp-(1->4)-beta-d-galp-(1->4)-beta-d-glcp-(1<->1')-cer
alpha-d-gal-(1->4)-beta-d-gal-(1->4)-beta-d-glc-(1<->1')-cer
cd77 antigen
p(k) antigen
gb3 globotriaosylceramide
burkitt's lymphoma antigen, bla-cd77
ganglioside ga3, o-alpha-d-galactopyranosyl-(1-4b)-
gal-1-4-gal-1-4-glc-cer
galactosyl(alpha1-4)galactosyl(beta1-4)glucosylceramide
shiga toxin receptor
gb3cer
globotriasoyl ceramide
vt2 receptor
slt-ii receptor
bla-cd77
alpha-d-galactosyl-(1->4)-beta-d-galactosyl-(1->4)-d-glucosyl-(1<->1)-ceramide
EPITOPE ID:153757
burkitt's lymphoma antigen
SCHEMBL182663
Q45435675

Research Excerpts

Overview

Globotriaosylceramide (Gb3) is a glycosphingolipid present in the plasma membrane that is the natural receptor of the bacterial Shiga toxin. It is a cell surface-expressed natural resistance factor for HIV infection, but, its expression in human T-cells remains unknown.

ExcerptReferenceRelevance
"Globotriaosylceramide (Gb3 or CD77) is a tumor-associated carbohydrate antigen implicated in several types of cancer that serves as a potential cancer marker for developing target-specific diagnosis and therapy. "( Repurposing the Pentameric B-Subunit of Shiga Toxin for Gb3-Targeted Immunotherapy of Colorectal Cancer by Rhamnose Conjugation.
Hong, H; Li, X; Liu, Z; Lu, Z; Pieters, RJ; Qin, X; Shi, J; Wu, Z; Zhou, Z, 2022
)
2.16
"Globotriaosylceramide (Gb3) is a glycosphingolipid present in the plasma membrane that is the natural receptor of the bacterial Shiga toxin. "( The effects of globotriaosylceramide tail saturation level on bilayer phases.
Chaban, VV; Ipsen, JH; Johannes, L; Khandelia, H; Pezeshkian, W; Shillcock, J, 2015
)
2.21
"Globotriaosylceramide (Gb3) is a glycosphingolipid present in cellular membranes that progressively accumulates in Fabry disease. "( Globotriaosylceramide inhibits iNKT-cell activation in a CD1d-dependent manner.
De Libero, G; Macedo, MF; Mori, L; Pereira, CS; Sa-Miranda, C, 2016
)
3.32
"Globotriaosylceramide (Gb(3)) is a cell surface-expressed natural resistance factor for HIV infection, but, its expression in human T-cells remains unknown. "( CD4(+) T-cells are unable to express the HIV natural resistance factor globotriosylceramide.
Binnington, B; Branch, DR; Kim, M; Lingwood, CA; Sakac, D, 2013
)
1.83
"Globotriaosylceramide (GL3) is a heterogeneous glycosphingolipid that is elevated in the blood plasma of patients diagnosed with Fabry disease. "( Globotriaosylceramide isoform profiles in human plasma by liquid chromatography-tandem mass spectrometry.
Araghi, S; Nelson, BC; Richards, SM; Roddy, T; Sung, CC; Thomas, JJ; Wilkens, D; Zhang, K, 2004
)
3.21
"Globotriaosylceramide is a neutral glycolipid containing the trihexoside Gal(alpha1-4)Gal(ss1-4)Glc(ss1-1') covalently bound to N-acylsphingosine. "( Rapid determination of urinary globotriaosylceramide isoform profiles by electrospray ionization mass spectrometry using stearoyl-d35-globotriaosylceramide as internal standard.
Breunig, F; Devrnja, D; Erwa, W; Fauler, G; Kotanko, P; Paschke, E; Plecko, B; Rechberger, GN, 2005
)
2.06
"Isoglobotriaosylceramide (iGb3) is a stimulatory antigen for a unique type of T cell, Natural Killer T cells. "( Sensitive quantitation of isoglobotriaosylceramide in the presence of isobaric components using electrospray ionization-ion trap mass spectrometry.
Levery, SB; Li, Y; Wang, PG; Xia, C; Zhou, D, 2008
)
1.26
"Globotriaosylceramide is a plasma membrane component, and the natural receptor for verotoxin derived from E."( Increased globotriaosylceramide on plasma membranes of synchronized familial dysautonomia cells. Verotoxin binding studies.
Boyd, B; Lingwood, C; Newbigging, J; Pereira, J; Strasberg, PM, 1994
)
1.41

Effects

ExcerptReferenceRelevance
"Globotriaosylceramide (Gb(3)) has been measured in urine of 35 male hemizygotes and 66 female heterozygotes for Fabry disease (FD). "( [Contribution of the measurement of globotriaosylceramide in urine to the diagnosis and follow-up of Fabry disease].
Froissart, R; Maire, I; Piraud, M, 2010
)
2.08

Toxicity

ExcerptReferenceRelevance
" Only the galactose blocked in alpha configuration provided a fine inhibition of 38-13 binding on BL Ramos cells and both alpha and beta-galactose allowed us to establish a clear distinction between the pathway entry of 38-13 IT in BL and non-BL cells; in close correlation with the 38-13 binding specificity the 38-13 IT cytotoxic effect in Ramos BL cells could also be prevented by alpha-Gal only, suggesting that this toxic action is probably mediated through the IT binding to Gb3 antigenic sites."( Comparison of inhibitory effect of galactose analogs on the binding and cytotoxicity of an anti-globotriaosylceramide monoclonal antibody coupled or not coupled to pokeweed antiviral protein.
Junqua, S; Le Pecq, JB; Mishal, Z; Wils, P, 1987
)
0.49
"Porcine and bovine aortic endothelial cells and human colonic adenocarcinoma cells were compared for their susceptibility to the toxic effect of purified Shiga-like toxin IIe (SLT-IIe), measured by the neutral red cytotoxicity assay."( Comparative cytotoxicity of purified Shiga-like toxin-IIe on porcine and bovine aortic endothelial and human colonic adenocarcinoma cells.
Boyd, B; Clarke, RC; Durette, A; Gyles, CL; Lingwood, C; MacLeod, DL; Valdivieso-Garcia, A, 1996
)
0.29
" This reduction in receptor binding corresponded to a 10(5)-fold reduction in the toxic activity of VT1 on a Vero cell monolayer."( Toxicity and immunogenicity of a verotoxin 1 mutant with reduced globotriaosylceramide receptor binding in rabbits.
Bast, DJ; Brunton, JL; Karmali, MA; Richardson, SE, 1997
)
0.53
"Proximal tubules were extremely sensitive to the cytotoxic effect of Stx-1 with an LD50 at least equal to, if not less than, that seen with Vero cells."( Cytotoxic effect of Shiga toxin-1 on human proximal tubule cells.
Hughes, AK; Kohan, DE; Stricklett, PK, 1998
)
0.3
" We found that while the site 1 and 2 mutants were modestly (two- to sevenfold) reduced in their ability to cause disease in BALB/c mice, the site 3 mutant, W34A, was as toxic as VT1."( Mouse toxicity and cytokine release by verotoxin 1 B subunit mutants.
Brunton, JL; Soltyk, AM; Wolski, VM, 2001
)
0.31
" The incidence of most treatment-related adverse events was similar in the two groups, with the exception of mild-to-moderate infusion reactions (i."( Safety and efficacy of recombinant human alpha-galactosidase A replacement therapy in Fabry's disease.
Caplan, L; Desnick, RJ; Eng, CM; Germain, DP; Guffon, N; Lee, P; Linthorst, GE; Waldek, S; Wilcox, WR, 2001
)
0.31
" Agalsidase beta was generally well tolerated; most treatment-related adverse events were mild or moderate infusion-associated reactions involving rigors, fever, or rhinitis."( Safety and efficacy of enzyme replacement therapy with agalsidase beta: an international, open-label study in pediatric patients with Fabry disease.
Germain, DP; Guffon, N; Lien, YH; Tsimaratos, M; Tylki-Szymanska, A; Vellodi, A; Wraith, JE, 2008
)
0.35
" The main aim was to evaluate the effects of Stx2 on EVT in order to understand the possible adverse effects that the toxin may have on trophoblast cells during early pregnancy."( Cytotoxic effects of Shiga toxin-2 on human extravillous trophoblast cell lines.
Amaral, MM; Damiano, AE; Ibarra, C; Reppetti, J; Sacerdoti, F; Scalise, ML, 2019
)
0.51
" Safety and efficacy evaluations included adverse drug reactions (ADRs), infusion-associated reactions and hypersensitivity reactions, and change in blood GL-3 level over time."( Long-term safety and efficacy of agalsidase beta in Japanese patients with Fabry disease: aggregate data from two post-authorization safety studies.
Hokugo, J; Suzuki, S; Tsurumi, M; Ueda, K, 2021
)
0.62

Pharmacokinetics

ExcerptReferenceRelevance
" The pharmacokinetic parameters of pediatric patients (19 boys, 5 girls, 6-18 years old; mean age, 11."( Enzyme replacement in Fabry disease: pharmacokinetics and pharmacodynamics of agalsidase alpha in children and adolescents.
Beck, M; Brady, RO; Clarke, JT; Loveday, KS; Mehta, A; Ries, M; Schiffmann, R; Whybra, C, 2007
)
0.34
"Pegunigalsidase alfa, a novel PEGylated, covalently crosslinked form of α-galactosidase A developed as enzyme replacement therapy (ERT) for Fabry disease (FD), was designed to increase plasma half-life and reduce immunogenicity, thereby enhancing efficacy compared with available products."( Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, provides sustained plasma concentrations and favorable pharmacodynamics: A 1-year Phase 1/2 clinical trial.
Alon, S; Atta, MG; Barisoni, L; Boyadjiev, SA; Brill-Almon, E; Charney, MR; Chertkoff, R; Colvin, RB; Giraldo, P; Goker-Alpan, O; Gonzalez, D; Holida, M; Hughes, D; Jennette, CJ; Maegawa, G; Nicholls, K; Paz, A; Rup, B; Schiffmann, R; Szlaifer, M; Tuffaha, A, 2019
)
0.51

Bioavailability

ExcerptReferenceRelevance
" Thus, a functional interplay between membrane Gb3 and MDR1 provides a more physiologically based approach to MDR1 regulation to increase the bioavailability of chemotherapeutic drugs."( Inhibition of multidrug resistance by adamantylgb3, a globotriaosylceramide analog.
Ackerley, C; Clarke, DM; De Rosa, MF; Ito, S; Lingwood, C; Wang, B, 2008
)
0.59
" Recent studies in the alpha-galactosidase A-knockout mouse suggested that a decrease in nitric oxide (NO) bioavailability may play a role in the abnormal thrombosis, atherogenesis, and vasorelaxation that are characteristic of these mice."( Decreased nitric oxide bioavailability in a mouse model of Fabry disease.
Byun, J; Kollmeyer, J; Park, JL; Pennathur, S; Shayman, JA; Shu, L, 2009
)
0.35
" These profound alterations in eNOS bioavailability at 8 mo of age were observed in parallel with high levels of 3-nitrotyrosine, suggesting increased reactive oxygen species along with eNOS uncoupling in this vascular bed."( Endothelial nitric oxide synthase uncoupling and microvascular dysfunction in the mesentery of mice deficient in α-galactosidase A.
Bodary, PF; Kang, JJ; Park, JL; Shayman, JA; Shu, L, 2014
)
0.4
" Lucerastat is an orally bioavailable inhibitor of glucosylceramide synthase (GCS) that is in late stage clinical development for Fabry disease."( Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types.
Garzotti, M; Groenen, PMA; Morand, O; Mühlemann, A; Probst, MR; Rickert, V; Üçeyler, N; Welford, RWD, 2018
)
0.72

Dosage Studied

ExcerptRelevanceReference
" However, the use of the anti-inflammatory drug dexamethasone and multiple dosing increased alpha-galactosidase A expression and resulted in significant reductions of GL-3 in all the organs with the exception of the kidney."( Partial correction of the alpha-galactosidase A deficiency and reduction of glycolipid storage in Fabry mice using synthetic vectors.
Cheng, SH; Desnick, RJ; Przybylska, M; Scheule, RK; Tousignant, JD; Wu, IH; Yew, NS; Zhao, H; Ziegler, RJ, 2004
)
0.32
" There were dose-response relations in the efficacy of each PUFA."( Contribution of polyunsaturated fatty acids to Shiga toxin cytotoxicity in human renal tubular epithelium-derived cells.
Sasaki, TK; Takita, T, 2006
)
0.33
"Twenty-two FD patients (20 men and 2 women) receiving dialysis or who had a history of kidney transplantation were treated with agalsidase alfa in an open label setting using the same dosing regimen given to patients without ESRD (0."( Safety and pharmacokinetics of agalsidase alfa in patients with Fabry disease and end-stage renal disease.
Barnett, N; Boyd, E; Crandall, K; Pastores, GM; Piersall, L; Whelan, A, 2007
)
0.34
"The same dosing regimen of agalsidase alfa may be safely administered to FD patients with ESRD as given to those without ESRD."( Safety and pharmacokinetics of agalsidase alfa in patients with Fabry disease and end-stage renal disease.
Barnett, N; Boyd, E; Crandall, K; Pastores, GM; Piersall, L; Whelan, A, 2007
)
0.34
"This 10-week study was conducted to determine the pharmacokinetics of varying doses of agalsidase alfa and evaluate the effect of dose and dosing frequency on plasma Gb3 levels."( The pharmacology of multiple regimens of agalsidase alfa enzyme replacement therapy for Fabry disease.
Bultas, J; Clarke, JT; Schiffmann, R; West, ML, 2007
)
0.34
" However, because plasma Gb3 is not a validated surrogate of disease severity in Fabry disease, further clinical study will be required to determine the optimal dosing regimen for providing maximal clinical benefit."( The pharmacology of multiple regimens of agalsidase alfa enzyme replacement therapy for Fabry disease.
Bultas, J; Clarke, JT; Schiffmann, R; West, ML, 2007
)
0.34
" In summary, long-term enzyme replacement therapy in young patients can result in complete globotriaocylceramide clearance of mesangial and glomerular endothelial cells across all dosage regimens, and clearance of podocyte inclusions is dose-dependent."( Agalsidase benefits renal histology in young patients with Fabry disease.
Bostad, L; Hirth, A; Houge, G; Larsen, KK; Svarstad, E; Tøndel, C; Vikse, BE, 2013
)
0.39
"Two trials compared different dosing schedules of agalsidase alfa."( Enzyme replacement therapy for Anderson-Fabry disease.
Barreto, FC; Barretti, P; Bazan, R; Camargo, SE; Carvalho, RP; El Dib, R; Gomaa, H, 2016
)
0.43
[information is derived through text-mining from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Pathways (1)

PathwayProteinsCompounds
Degradation pathway of sphingolipids, including diseases93

Research

Studies (704)

TimeframeStudies, This Drug (%)All Drugs %
pre-199040 (5.68)18.7374
1990's128 (18.18)18.2507
2000's262 (37.22)29.6817
2010's227 (32.24)24.3611
2020's47 (6.68)2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Market Indicators

Research Demand Index: 38.80

According to the monthly volume, diversity, and competition of internet searches for this compound, as well the volume and growth of publications, there is estimated to be strong demand-to-supply ratio for research on this compound.

MetricThis Compound (vs All)
Research Demand Index38.80 (24.57)
Research Supply Index6.64 (2.92)
Research Growth Index4.98 (4.65)
Search Engine Demand Index58.83 (26.88)
Search Engine Supply Index2.00 (0.95)

This Compound (38.80)

All Compounds (24.57)

Study Types

Publication TypeThis drug (%)All Drugs (%)
Trials32 (4.37%)5.53%
Reviews89 (12.16%)6.00%
Case Studies34 (4.64%)4.05%
Observational1 (0.14%)0.25%
Other576 (78.69%)84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]