J 2021

The transcriptional landscape of Shh medulloblastoma

SKOWRON, P., H. FAROOQ, F. M. G. CAVALLI, A. S. MORRISSY, M. LY et. al.

Základní údaje

Originální název

The transcriptional landscape of Shh medulloblastoma

Autoři

SKOWRON, P., H. FAROOQ, F. M. G. CAVALLI, A. S. MORRISSY, M. LY, L. D. HENDRIKSE, E. Y. WANG, H. DJAMBAZIAN, H. ZHU, K. L. MUNGALL, Q. M. TRINH, T. N. ZHENG, S. Z. DAI, A. S. G. STUCKLIN, M. C. VLADOIU, V. FONG, B. L. HOLGADO, C. NOR, X. C. WU, D. ABD-RABBO, P. BERUBE, Y. C. WANG, B. LUU, R. A. SUAREZ, A. RASTAN, A. H. GILLMOR, J. J. Y. LEE, X. Y. ZHANG, C. DANIELS, P. DIRKS, D. MALKIN, E. BOUFFET, U. TABORI, J. LOUKIDES, F. P. DOZ, F. BOURDEAUT, O. O. DELATTRE, J. MASLIAH-PLANCHON, O. AYRAULT, S. K. KIM, D. MEYRONET, W. A. GRAJKOWSKA, C. G. CARLOTTI, C. DE TORRES, J. MORA, C. G. EBERHART, E. G. VAN MEIR, T. KUMABE, P. J. FRENCH, J. M. KROS, N. JABADO, B. LACH, I. F. POLLACK, R. L. HAMILTON, A. A. N. RAO, C. GIANNINI, J. M. OLSON, L. BOGNAR, A. KLEKNER, Karel ZITTERBART (203 Česká republika, domácí), J. J. PHILLIPS, R. C. THOMPSON, M. K. COOPER, J. B. RUBIN, L. M. LIAU, M. GARAMI, P. HAUSER, K. K. W. LI, H. K. NG, W. S. POON, G. Y. GILLESPIE, J. A. CHAN, S. JUNG, R. E. MCLENDON, E. M. THOMPSON, D. ZAGZAG, R. VIBHAKAR, Y. S. RA, M. L. GARRE, U. SCHULLER, T. SHOFUDA, C. C. FARIA, E. LOPEZ-AGUILAR, G. ZADEH, C. C. HUI, V. RAMASWAMY, S. D. BAILEY, S. J. JONES, A. J. MUNGALL, R. A. MOORE, J. A. CALARCO, L. D. STEIN, G. D. BADER, J. REIMAND, J. RAGOUSSIS, W. A. WEISS, M. A. MARRA, H. SUZUKI (garant) a MD TAYLOR

Vydání

Nature Communications, London, Nature Publishing Group, 2021, 2041-1723

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

10700 1.7 Other natural sciences

Stát vydavatele

Německo

Utajení

není předmětem státního či obchodního tajemství

Odkazy

Impakt faktor

Impact factor: 17.694

Kód RIV

RIV/00216224:14110/21:00122127

Organizační jednotka

Lékařská fakulta

UT WoS

000631928500006

Klíčová slova anglicky

Shh medulloblastoma; transcriptional landscape

Štítky

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 19. 8. 2021 11:50, Mgr. Tereza Miškechová

Anotace

V originále

Sonic hedgehog medulloblastoma encompasses a clinically and molecularly diverse group of cancers of the developing central nervous system. Here, we use unbiased sequencing of the transcriptome across a large cohort of 250 tumors to reveal differences among molecular subtypes of the disease, and demonstrate the previously unappreciated importance of non-coding RNA transcripts. We identify alterations within the cAMP dependent pathway (GNAS, PRKAR1A) which converge on GLI2 activity and show that 18% of tumors have a genetic event that directly targets the abundance and/or stability of MYCN. Furthermore, we discover an extensive network of fusions in focally amplified regions encompassing GLI2, and several loss-of-function fusions in tumor suppressor genes PTCH1, SUFU and NCOR1. Molecular convergence on a subset of genes by nucleotide variants, copy number aberrations, and gene fusions highlight the key roles of specific pathways in the pathogenesis of Sonic hedgehog medulloblastoma and open up opportunities for therapeutic intervention. Sonic Hedgehog medulloblastoma (Shh-MB) comprises four subtypes each with distinct clinical traits. Here the authors characterize the genome, transcriptome, and methylome of Shh-MB subtypes, revealing a complex fusion landscape and the molecular convergence of MYCN and cAMP signaling pathways.