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Truncated BRPF1 Cooperates With Smoothened to Promote Adult Shh Medulloblastoma

Truncated BRPF1 Cooperates With Smoothened to Promote Adult Shh Medulloblastoma

Giuseppe Aiello, Claudio Ballabio, Riccardo Ruggeri, Luca Fagnocchi, Marica Anderle, Ilaria Morassut, Davide Caron, Francesca Garilli, Francesca Gianno, Felice Giangaspero, Silvano Piazza, Alessandro Romanel, etc.

Cell Rep. 2019 Dec 17;29(12):4036-4052.e10.

PMID: 31851932

Abstract:

The transition of neural progenitors to differentiated postmitotic neurons is mainly considered irreversible in physiological conditions. In the present work, we show that Shh pathway activation through SmoM2 expression promotes postmitotic neurons dedifferentiation, re-entering in the cell cycle and originating medulloblastoma in vivo. Notably, human adult patients present inactivating mutations of the chromatin reader BRPF1 that are associated with SMO mutations and absent in pediatric and adolescent patients. Here, we found that truncated BRPF1 protein, as found in human adult patients, is able to induce medulloblastoma in adult mice upon SmoM2 activation. Indeed, postmitotic neurons re-entered the cell cycle and proliferated as a result of chromatin remodeling of neurons by BRPF1. Our model of brain cancer explains the onset of a subset of human medulloblastoma in adult individuals where granule neuron progenitors are no longer present.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
IAR42416124	BRPF1 (627-746) human			Price

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