% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Hutter:128815,
author = {S. Hutter$^*$ and R. M. Piro$^*$ and S. M. Waszak and H.
Kehrer-Sawatzki and R. E. Friedrich and A. Lassaletta and O.
Witt$^*$ and J. O. Korbel and P. Lichter$^*$ and M. U.
Schuhmann$^*$ and S. Pfister$^*$ and U. Tabori and V. F.
Mautner and D. Jones$^*$},
title = {{N}o correlation between {NF}1 mutation position and risk
of optic pathway glioma in 77 unrelated {NF}1 patients.},
journal = {Human genetics},
volume = {135},
number = {5},
issn = {1432-1203},
address = {Berlin},
publisher = {Springer},
reportid = {DKFZ-2017-04828},
pages = {469 - 475},
year = {2016},
abstract = {Neurofibromatosis type 1 (NF1) is a common monogenic
disorder whereby affected individuals are predisposed to
developing CNS tumors, including optic pathway gliomas
(OPGs, occurring in ~15 to $20 \%$ of cases). So far, no
definite genotype-phenotype correlation determining NF1
patients at risk for tumor formation has been described,
although enrichment for mutations in the 5' region of the
NF1 gene in OPG patients has been suggested. We used whole
exome sequencing, targeted sequencing, and copy number
analysis to screen 77 unrelated NF1 patients with (n = 41)
or without (n = 36; age ≥10 years) optic pathway
glioma for germline NF1 alterations. We identified germline
NF1 mutations in 69 of 77 patients $(90 \%),$ but no
genotype-phenotype correlation was observed. Our data using
a larger patient cohort did not confirm the previously
reported clustering of mutations in the 5' region of the NF1
gene in patients with OPG. Thus, NF1 mutation location
should not currently be used as a clinical criterion to
assess the risk of developing OPGs.},
keywords = {Neurofibromin 1 (NLM Chemicals)},
cin = {B062 / B060 / G340 / L101 / L801},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)B060-20160331 /
I:(DE-He78)G340-20160331 / I:(DE-He78)L101-20160331 /
I:(DE-He78)L801-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:26969325},
doi = {10.1007/s00439-016-1646-x},
url = {https://inrepo02.dkfz.de/record/128815},
}