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@ARTICLE{Johann:154561,
author = {P. D. Johann$^*$},
title = {{I}nvited {R}eview: {D}ysregulation of chromatin
remodellers in paediatric brain tumours - {SMARCB}1 and
beyond.},
journal = {Neuropathology $\&$ applied neurobiology},
volume = {46},
number = {1},
issn = {1365-2990},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2020-00856},
pages = {57 - 72},
year = {2020},
note = {#LA:B062#Invited Review},
abstract = {Mutations in chromatin remodelling genes occur in
approximately $25\%$ of all human tumours (Kadoch et al. Nat
Genet 45: 592-601, 2013). The spectrum of alterations is
broad and comprises single nucleotide variants,
insertion/deletions and more complex structural variations.
The single most often affected remodelling complex is the
SWI/SNF complex (SWItch/sucrose non-fermentable). In the
field of paediatric neuro-oncology, the spectrum of affected
genes implicated in epigenetic remodelling is narrower with
SMARCB1 and SMARCA4 being the most frequent. The low
mutation frequencies in many of the SWI/SNF mutant entities
underline the fact that perturbed chromatin remodelling is
the most salient factor in tumourigenesis and could thus be
a potential therapeutic opportunity. Here, I review the
genetic basis of aberrant chromatin remodelling in
paediatric brain tumours and discuss their impact on the
epigenome in the respective entities, mainly
medulloblastomas and rhabdoid tumours.},
subtyp = {Review Article},
cin = {B062 / HD01},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:32307752},
doi = {10.1111/nan.12616},
url = {https://inrepo02.dkfz.de/record/154561},
}