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@ARTICLE{Billaud:306176,
author = {A. Billaud and G. Figlioli and C. Mooser and I. Casamassima
and V. Azzoni and J. Srivatsa and M. Colombo and L. Caleca
and T. U. Ahearn and I. L. Andrulis and A. C. Antoniou and
M. W. Beckmann and S. Behrens$^*$ and M. Bermisheva and N.
V. Bogdanova and M. K. Bolla and B. Bonanni and T. Brüning
and N. J. Camp and A. Campbell and J. E. Castelao and M. H.
Cessna and J. Chang-Claude$^*$ and K. Czene and J. Dennis
and P. Devilee and T. Dörk and A. M. Dunning and M.
Eriksson and D. G. Evans and P. A. Fasching and J. D.
Figueroa and M. Gabrielson and M. Gago-Dominguez and A.
González-Neira and P. Guénel and A. Hadjisavvas and E.
Hahnen and U. Hamann$^*$ and P. Hillemanns and A.
Hollestelle and M. J. Hooning and R. Hoppe and A. Howell and
A. Jakubowska and V. N. Kristensen and J. Lubiński and M.
Lush and S. Manoukian and D. Mavroudis and R. L. Milne and
A. M. Mulligan and W. G. Newman and N. Obi and M. I.
Panayiotidis and G. Pita and M. U. Rashid$^*$ and V. Rhenius
and E. Saloustros and E. J. Sawyer and R. K. Schmutzler and
M. Shah and M. C. Southey and A. B. Spurdle and I. Tomlinson
and T. Truong and Q. Wang and C. Wendt and P. L. Auer and N.
J. Boddicker and C. Bodelon and E. S. Burnside and F. Chen
and F. J. Couch and S. M. Domchek and H. A. Eliassen and C.
Haiman and J. M. Hodge and C. Hu and H. Huang and S.
Lindstrom and M. E. Martinez and K. L. Nathanson and S. L.
Neuhausen and K. M. O'Brien and J. E. Olson and J. R. Palmer
and A. V. Patel and K. J. Ruddy and D. P. Sandler and L. R.
Teras and C. R. Weinberg and J. N. Weitzel and S. J. Winham
and S. Yadav and S. Yao and G. Zirpoli and M. Janatova and
Z. Kleibl and P. Kleiblova and J. Soukupova and Q. Zhao and
L. Devereux and P. A. James and I. G. Campbell and T.
Nguyen-Dumont and J. G. Dowty and N. Andrieu and F. Lesueur
and D. Stoppa-Lyonnet and M. d. l. Hoya and P. Radice and C.
S. Sørensen and P. Peterlongo},
collaboration = {NBCS Collaborators and k. Investigators and C. consortium
and GENESIS},
title = {{L}arge-scale meta-analysis and precision functional assays
identify {FANCM} regions in which {PTV}s confer different
risks for {ER}-negative and triple-negative breast cancer.},
journal = {The breast},
volume = {85},
issn = {0960-9776},
address = {Amsterdam [u.a.]},
publisher = {Elsevier},
reportid = {DKFZ-2025-02416},
pages = {104619},
year = {2025},
abstract = {The breast cancer risk conferred by germline protein
truncating variants (PTVs) in known and putative breast
cancer genes has been extensively investigated. However, the
effect of FANCM PTVs on breast cancer risk remains unclear.
Our previous clinical, genetic and functional results on the
N-terminal p.Arg658∗ and the two C-terminal p.Gln1701∗
and p.Gly1906Alafs∗12 variants suggested that FANCM PTVs
may confer different risks for ER-negative (ER-neg) and
triple-negative (TN) breast cancer subtypes. Here, we
performed meta-analyses of seven studies totaling 144 681
breast cancer cases and 123 632 controls. FANCM PTVs were
tested for association with breast cancer risk overall and
the disease clinical subtypes by single variant and burden
analyses. Two CRISPR-Cas9-based functional assays were also
conducted to test the fitness of cells after knock-in of the
p.Arg658∗, p.Gln1701∗ and p.Gly1906Alafs∗12 PTVs and
the sensitivity of different FANCM regions to genome
editing. Our results suggest that the N-terminal FANCM
region upstream of p.Tyr725 harbors essential functions,
whereas downstream regions appear dispensable. This is
supported by our genetic data which indicate that all FANCM
PTVs, excluding the two C-terminal p.Gln1701∗ and
p.Gly1906Alafs∗12, are associated with an increased risk
of ER-neg (OR = 1.41, P = 0.023) and TN (OR = 1.64, P =
0.0023). Notably, PTVs upstream of AA position 670 are
associated with a moderate risk of developing TN breast
cancer, and that even when the p.Arg658∗ carriers were
excluded from the analysis. Importantly, our results confirm
previous data indicating that p.Arg658∗ carriers are at
moderate risk of developing ER-neg (OR = 2.08, P = 0.030)
and TN (OR = 3.26; P = 0.0034), whereas carriers of
p.Gln1701∗ and p.Gly1906Alafs∗12 should not be
considered at increased risk. Our data are useful for
counseling carriers of FANCM PTVs, but further analyses are
warranted to obtain more precise risk estimates.},
keywords = {Breast cancer risk (Other) / CRISPR-Cas9 (Other) / FANCM
(Other) / Gene editing (Other) / Genetic predisposition
(Other) / Meta-analysis (Other)},
cin = {C020 / B072},
ddc = {610},
cid = {I:(DE-He78)C020-20160331 / I:(DE-He78)B072-20160331},
pnm = {313 - Krebsrisikofaktoren und Prävention (POF4-313)},
pid = {G:(DE-HGF)POF4-313},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41223770},
doi = {10.1016/j.breast.2025.104619},
url = {https://inrepo02.dkfz.de/record/306176},
}