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Comment
. 2014 Jan;46(1):2-3.
doi: 10.1038/ng.2857.

A developmental program drives aggressive embryonal brain tumors

Tenley C Archer  1 , Scott L Pomeroy  1
Affiliations
Comment

A developmental program drives aggressive embryonal brain tumors

Tenley C Archer et al. Nat Genet. 2014 Jan.

Abstract

Embryonal tumors with multilayered rosettes (ETMRs) are primitive neuroectodermal tumors arising in infants. A new study shows that these tumors are universally driven by fusion of the promoter of a gene with brain-specific expression, TTYH1, to C19MC, the largest human microRNA cluster, activating a fetal neural development program.

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  • Fusion of TTYH1 with the C19MC microRNA cluster drives expression of a brain-specific DNMT3B isoform in the embryonal brain tumor ETMR.
    Kleinman CL, Gerges N, Papillon-Cavanagh S, Sin-Chan P, Pramatarova A, Quang DA, Adoue V, Busche S, Caron M, Djambazian H, Bemmo A, Fontebasso AM, Spence T, Schwartzentruber J, Albrecht S, Hauser P, Garami M, Klekner A, Bognar L, Montes JL, Staffa A, Montpetit A, Berube P, Zakrzewska M, Zakrzewski K, Liberski PP, Dong Z, Siegel PM, Duchaine T, Perotti C, Fleming A, Faury D, Remke M, Gallo M, Dirks P, Taylor MD, Sladek R, Pastinen T, Chan JA, Huang A, Majewski J, Jabado N. Kleinman CL, et al. Nat Genet. 2014 Jan;46(1):39-44. doi: 10.1038/ng.2849. Epub 2013 Dec 8. Nat Genet. 2014. PMID: 24316981

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