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@ARTICLE{Srivastava:154585,
author = {A. Srivastava$^*$ and S. Giangiobbe$^*$ and A. Kumar$^*$
and N. Paramasivam$^*$ and D. Dymerska and W. Behnisch and
M. Witzens-Harig and J. Lubinski and K. Hemminki$^*$ and A.
Försti$^*$ and O. R. Bandapalli$^*$},
title = {{I}dentification of {F}amilial {H}odgkin {L}ymphoma
{P}redisposing {G}enes {U}sing {W}hole {G}enome
{S}equencing.},
journal = {Frontiers in Bioengineering and Biotechnology},
volume = {8},
issn = {2296-4185},
address = {Lausanne},
publisher = {Frontiers Media},
reportid = {DKFZ-2020-00864},
pages = {179},
year = {2020},
note = {#EA:B062#EA:C050##LA:C050#LA:B062#},
abstract = {Hodgkin lymphoma (HL) is a lymphoproliferative malignancy
of B-cell origin that accounts for $10\%$ of all lymphomas.
Despite evidence suggesting strong familial clustering of
HL, there is no clear understanding of the contribution of
genes predisposing to HL. In this study, whole genome
sequencing (WGS) was performed on 7 affected and 9
unaffected family members from three HL-prone families and
variants were prioritized using our Familial Cancer Variant
Prioritization Pipeline (FCVPPv2). WGS identified a total of
98,564, 170,550, and 113,654 variants which were reduced by
pedigree-based filtering to 18,158, 465, and 26,465 in
families I, II, and III, respectively. In addition to
variants affecting amino acid sequences, variants in
promoters, enhancers, transcription factors binding sites,
and microRNA seed sequences were identified from upstream,
downstream, 5' and 3' untranslated regions. A panel of 565
cancer predisposing and other cancer-related genes and of
2,383 potential candidate HL genes were also screened in
these families to aid further prioritization. Pathway
analysis of segregating genes with Combined Annotation
Dependent Depletion Tool (CADD) scores >20 was performed
using Ingenuity Pathway Analysis software which implicated
several candidate genes in pathways involved in B-cell
activation and proliferation and in the network of 'Cancer,
Hematological disease and Immunological Disease.' We used
the FCVPPv2 for further in silico analyses and prioritized
45 coding and 79 non-coding variants from the three
families. Further literature-based analysis allowed us to
constrict this list to one rare germline variant each in
families I and II and two in family III. Functional studies
were conducted on the candidate from family I in a previous
study, resulting in the identification and functional
validation of a novel heterozygous missense variant in the
tumor suppressor gene DICER1 as potential HL predisposition
factor. We aim to identify the individual genes responsible
for predisposition in the remaining two families and will
functionally validate these in further studies.},
cin = {C050 / B062 / HD01 / C020},
ddc = {570},
cid = {I:(DE-He78)C050-20160331 / I:(DE-He78)B062-20160331 /
I:(DE-He78)HD01-20160331 / I:(DE-He78)C020-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:32211398},
pmc = {pmc:PMC7067901},
doi = {10.3389/fbioe.2020.00179},
url = {https://inrepo02.dkfz.de/record/154585},
}