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@ARTICLE{Srivastava:300275,
author = {A. Srivastava$^*$ and D. Skopelitou$^*$ and B. Miao$^*$ and
S. Giagiobbe$^*$ and N. Paramasivam and A. Kumar$^*$ and C.
Diquigiovanni and E. Bonora and O. R. Bandapalli$^*$ and A.
Försti$^*$ and K. Hemminki$^*$},
title = {{P}rioritization of predisposition genes for familial
non-medullary thyroid cancer by whole-genome sequencing.},
journal = {European journal of endocrinology},
volume = {192},
number = {4},
issn = {0001-5598},
address = {Oxford},
publisher = {Oxford University Press},
reportid = {DKFZ-2025-00728},
pages = {398 - 407},
year = {2025},
note = {#EA:C050#LA:C050#LA:B062# ; geteilte Letztautorenschaft},
abstract = {Thyroid cancer (TC) is the most common endocrine
malignancy, with $90\%-95\%$ of the cases representing
non-medullary thyroid cancer (NMTC). Familial cases account
only for a few of all cases and the underlying genetic
causes are still poorly understood.We whole-genome sequenced
affected and unaffected members of an Italian NMTC family
and applied our in-house developed Familial Cancer Variant
Prioritization Pipeline (FCVPPv2) which prioritized 12
coding variants. We refined this selection using the VarSome
American College of Medical Genetics and Genomics (ACMG)
implementation, SNAP2 predictions and further in silico
scores.We prioritized 4 possibly pathogenic variants in 4
genes including Ret proto-oncogene (RET), polypeptide
N-acetylgalactosaminyltransferase 10 (GALNT10), ubinuclein-1
(UBN1), and prostaglandin I2 receptor (PTGIR). The role of
RET point mutations in medullary thyroid carcinoma is well
established. Similarly, somatic rearrangements of RET are
known in papillary TC, a specific histotype of NMTC. In
contrast to RET, no germline variants in PTGIR, GALNT10, or
UBN1 have been linked to the development of TC to date.
However, alterations in these genes have been shown to
affect pathways related to cell proliferation, apoptosis,
growth, and differentiation, as well as posttranslational
modification and gene regulation. A thorough review of the
available literature together with computational evidence
supported the interpretation of the 4 shortlisted variants
as possibly disease-causing in this family.Our results
implicate the first germline variant in RET in a family with
NMTC as well as the first germline variants in PTGIR,
GALNT10, and UBN1 in TC.},
keywords = {Humans / Thyroid Neoplasms: genetics / Genetic
Predisposition to Disease: genetics / Proto-Oncogene Mas /
Male / Female / N-Acetylgalactosaminyltransferases: genetics
/ Proto-Oncogene Proteins c-ret: genetics / Pedigree / Whole
Genome Sequencing: methods / Middle Aged / Adult /
Polypeptide N-acetylgalactosaminyltransferase / Carcinoma,
Neuroendocrine: genetics / GALNT10 (Other) / PTGIR (Other) /
RET (Other) / UBN1 (Other) / familial non–medullary
thyroid cancer (Other) / germline variant (Other) /
whole-genome sequencing (Other) / Proto-Oncogene Mas (NLM
Chemicals) / N-Acetylgalactosaminyltransferases (NLM
Chemicals) / MAS1 protein, human (NLM Chemicals) /
Proto-Oncogene Proteins c-ret (NLM Chemicals) / RET protein,
human (NLM Chemicals) / Polypeptide
N-acetylgalactosaminyltransferase (NLM Chemicals)},
cin = {C050 / B062 / Z999 / A360 / HD01},
ddc = {610},
cid = {I:(DE-He78)C050-20160331 / I:(DE-He78)B062-20160331 /
I:(DE-He78)Z999-20160331 / I:(DE-He78)A360-20160331 /
I:(DE-He78)HD01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40177881},
doi = {10.1093/ejendo/lvaf045},
url = {https://inrepo02.dkfz.de/record/300275},
}