Matches in SemOpenAlex for { <https://semopenalex.org/work/W3128016821> ?p ?o ?g. }
- W3128016821 endingPage "520" @default.
- W3128016821 startingPage "520" @default.
- W3128016821 abstract "The ganglioside GD2 is an important target in childhood cancer. Nevertheless, the only therapy targeting GD2 that is approved to date is the monoclonal antibody dinutuximab, which is used in the therapy of neuroblastoma. The relevance of GD2 as a target in other tumor entities remains to be elucidated. Here, we analyzed the expression of GD2 in different pediatric tumor entities by flow cytometry and tested two approaches for targeting GD2. H3K27M-mutant diffuse midline glioma (H3K27M-mutant DMG) samples showed the highest expression of GD2 with all cells strongly positive for the antigen. Ewing’s sarcoma (ES) samples also showed high expression, but displayed intra- and intertumor heterogeneity. Osteosarcoma had low to intermediate expression with a high percentage of GD2-negative cells. Dinutuximab beta in combination with irinotecan and temozolomide was used to treat a five-year-old girl with refractory ES. Disease control lasted over 12 months until a single partially GD2-negative intracranial metastasis was detected. In order to target GD2 in H3K27M-mutant DMG, we blocked ganglioside synthesis via eliglustat, since dinutuximab cannot cross the blood–brain barrier. Eliglustat is an inhibitor of glucosylceramide synthase, and it is used for treating children with Gaucher’s disease. Eliglustat completely inhibited the proliferation of primary H3K27M-mutant DMG cells in vitro. In summary, our data provide evidence that dinutuximab might be effective in tumors with high GD2 expression. Moreover, disrupting the ganglioside metabolism in H3K27M-mutant DMG could open up a new therapeutic option for this highly fatal cancer." @default.
- W3128016821 created "2021-02-15" @default.
- W3128016821 creator A5004269135 @default.
- W3128016821 creator A5009280452 @default.
- W3128016821 creator A5011043287 @default.
- W3128016821 creator A5019220658 @default.
- W3128016821 creator A5019268274 @default.
- W3128016821 creator A5022305423 @default.
- W3128016821 creator A5035320359 @default.
- W3128016821 creator A5041040348 @default.
- W3128016821 creator A5047110708 @default.
- W3128016821 creator A5047133968 @default.
- W3128016821 creator A5047886974 @default.
- W3128016821 creator A5058426254 @default.
- W3128016821 creator A5063393972 @default.
- W3128016821 creator A5075160856 @default.
- W3128016821 creator A5076734902 @default.
- W3128016821 creator A5080611953 @default.
- W3128016821 creator A5084919495 @default.
- W3128016821 creator A5086434383 @default.
- W3128016821 date "2021-01-29" @default.
- W3128016821 modified "2023-09-26" @default.
- W3128016821 title "Exploiting Gangliosides for the Therapy of Ewing’s Sarcoma and H3K27M-Mutant Diffuse Midline Glioma" @default.
- W3128016821 cites W1513821330 @default.
- W3128016821 cites W1657723498 @default.
- W3128016821 cites W1947177866 @default.
- W3128016821 cites W1951179504 @default.
- W3128016821 cites W1970247466 @default.
- W3128016821 cites W1971736106 @default.
- W3128016821 cites W1973195150 @default.
- W3128016821 cites W1974081722 @default.
- W3128016821 cites W1975044418 @default.
- W3128016821 cites W1985774086 @default.
- W3128016821 cites W1986399662 @default.
- W3128016821 cites W2004504668 @default.
- W3128016821 cites W2017903224 @default.
- W3128016821 cites W2027442953 @default.
- W3128016821 cites W2041620451 @default.
- W3128016821 cites W2047902191 @default.
- W3128016821 cites W2050780415 @default.
- W3128016821 cites W2060799642 @default.
- W3128016821 cites W2067431214 @default.
- W3128016821 cites W2088282752 @default.
- W3128016821 cites W2095970177 @default.
- W3128016821 cites W2096266806 @default.
- W3128016821 cites W2103460784 @default.
- W3128016821 cites W2105687233 @default.
- W3128016821 cites W2114101532 @default.
- W3128016821 cites W2128125459 @default.
- W3128016821 cites W2129148702 @default.
- W3128016821 cites W2130465135 @default.
- W3128016821 cites W2134121541 @default.
- W3128016821 cites W2142287133 @default.
- W3128016821 cites W2150636854 @default.
- W3128016821 cites W2161577092 @default.
- W3128016821 cites W2179454156 @default.
- W3128016821 cites W2300857522 @default.
- W3128016821 cites W2319781178 @default.
- W3128016821 cites W2346144030 @default.
- W3128016821 cites W2468936663 @default.
- W3128016821 cites W2503515884 @default.
- W3128016821 cites W2606835938 @default.
- W3128016821 cites W2617103357 @default.
- W3128016821 cites W2618869560 @default.
- W3128016821 cites W2751476134 @default.
- W3128016821 cites W2755922861 @default.
- W3128016821 cites W2759328813 @default.
- W3128016821 cites W2763302201 @default.
- W3128016821 cites W2766319190 @default.
- W3128016821 cites W2770704851 @default.
- W3128016821 cites W2796163387 @default.
- W3128016821 cites W2796436502 @default.
- W3128016821 cites W2803040262 @default.
- W3128016821 cites W2804564451 @default.
- W3128016821 cites W2808090012 @default.
- W3128016821 cites W2811247652 @default.
- W3128016821 cites W2884159204 @default.
- W3128016821 cites W2889489530 @default.
- W3128016821 cites W2897377013 @default.
- W3128016821 cites W2900463817 @default.
- W3128016821 cites W2900717255 @default.
- W3128016821 cites W2917149656 @default.
- W3128016821 cites W2937119895 @default.
- W3128016821 cites W2950350154 @default.
- W3128016821 cites W2952481429 @default.
- W3128016821 cites W2953047993 @default.
- W3128016821 cites W2984919788 @default.
- W3128016821 cites W3001348553 @default.
- W3128016821 cites W3013424806 @default.
- W3128016821 cites W3028603350 @default.
- W3128016821 cites W3041005476 @default.
- W3128016821 cites W3041156479 @default.
- W3128016821 cites W3047432100 @default.
- W3128016821 cites W3080896648 @default.
- W3128016821 cites W3107142932 @default.
- W3128016821 cites W4232313822 @default.
- W3128016821 cites W4234163739 @default.
- W3128016821 doi "https://doi.org/10.3390/cancers13030520" @default.