000306176 001__ 306176 000306176 005__ 20251203110707.0 000306176 0247_ $$2doi$$a10.1016/j.breast.2025.104619 000306176 0247_ $$2pmid$$apmid:41223770 000306176 0247_ $$2ISSN$$a0960-9776 000306176 0247_ $$2ISSN$$a1532-3080 000306176 037__ $$aDKFZ-2025-02416 000306176 041__ $$aEnglish 000306176 082__ $$a610 000306176 1001_ $$aBillaud, Amandine$$b0 000306176 245__ $$aLarge-scale meta-analysis and precision functional assays identify FANCM regions in which PTVs confer different risks for ER-negative and triple-negative breast cancer. 000306176 260__ $$aAmsterdam [u.a.]$$bElsevier$$c2025 000306176 3367_ $$2DRIVER$$aarticle 000306176 3367_ $$2DataCite$$aOutput Types/Journal article 000306176 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1764756401_1331673 000306176 3367_ $$2BibTeX$$aARTICLE 000306176 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000306176 3367_ $$00$$2EndNote$$aJournal Article 000306176 520__ $$aThe breast cancer risk conferred by germline protein truncating variants (PTVs) in known and putative breast cancer genes has been extensively investigated. However, the effect of FANCM PTVs on breast cancer risk remains unclear. Our previous clinical, genetic and functional results on the N-terminal p.Arg658∗ and the two C-terminal p.Gln1701∗ and p.Gly1906Alafs∗12 variants suggested that FANCM PTVs may confer different risks for ER-negative (ER-neg) and triple-negative (TN) breast cancer subtypes. Here, we performed meta-analyses of seven studies totaling 144 681 breast cancer cases and 123 632 controls. FANCM PTVs were tested for association with breast cancer risk overall and the disease clinical subtypes by single variant and burden analyses. Two CRISPR-Cas9-based functional assays were also conducted to test the fitness of cells after knock-in of the p.Arg658∗, p.Gln1701∗ and p.Gly1906Alafs∗12 PTVs and the sensitivity of different FANCM regions to genome editing. Our results suggest that the N-terminal FANCM region upstream of p.Tyr725 harbors essential functions, whereas downstream regions appear dispensable. This is supported by our genetic data which indicate that all FANCM PTVs, excluding the two C-terminal p.Gln1701∗ and p.Gly1906Alafs∗12, are associated with an increased risk of ER-neg (OR = 1.41, P = 0.023) and TN (OR = 1.64, P = 0.0023). Notably, PTVs upstream of AA position 670 are associated with a moderate risk of developing TN breast cancer, and that even when the p.Arg658∗ carriers were excluded from the analysis. Importantly, our results confirm previous data indicating that p.Arg658∗ carriers are at moderate risk of developing ER-neg (OR = 2.08, P = 0.030) and TN (OR = 3.26; P = 0.0034), whereas carriers of p.Gln1701∗ and p.Gly1906Alafs∗12 should not be considered at increased risk. Our data are useful for counseling carriers of FANCM PTVs, but further analyses are warranted to obtain more precise risk estimates. 000306176 536__ $$0G:(DE-HGF)POF4-313$$a313 - Krebsrisikofaktoren und Prävention (POF4-313)$$cPOF4-313$$fPOF IV$$x0 000306176 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de 000306176 650_7 $$2Other$$aBreast cancer risk 000306176 650_7 $$2Other$$aCRISPR-Cas9 000306176 650_7 $$2Other$$aFANCM 000306176 650_7 $$2Other$$aGene editing 000306176 650_7 $$2Other$$aGenetic predisposition 000306176 650_7 $$2Other$$aMeta-analysis 000306176 7001_ $$aFiglioli, Gisella$$b1 000306176 7001_ $$aMooser, Clémence$$b2 000306176 7001_ $$aCasamassima, Irene$$b3 000306176 7001_ $$aAzzoni, Violette$$b4 000306176 7001_ $$aSrivatsa, Jahnavi$$b5 000306176 7001_ $$aColombo, Mara$$b6 000306176 7001_ $$aCaleca, Laura$$b7 000306176 7001_ $$aAhearn, Thomas U$$b8 000306176 7001_ $$aAndrulis, Irene L$$b9 000306176 7001_ $$aAntoniou, Antonis C$$b10 000306176 7001_ $$aBeckmann, Matthias W$$b11 000306176 7001_ $$0P:(DE-He78)6b04712f3afe72044d496a25505cb1ea$$aBehrens, Sabine$$b12$$udkfz 000306176 7001_ $$aBermisheva, Marina$$b13 000306176 7001_ $$aBogdanova, Natalia V$$b14 000306176 7001_ $$aBolla, Manjeet K$$b15 000306176 7001_ $$aBonanni, Bernardo$$b16 000306176 7001_ $$aBrüning, Thomas$$b17 000306176 7001_ $$aCamp, Nicola J$$b18 000306176 7001_ $$aCampbell, Archie$$b19 000306176 7001_ $$aCastelao, Jose E$$b20 000306176 7001_ $$aCessna, Melissa H$$b21 000306176 7001_ $$0P:(DE-He78)c259d6cc99edf5c7bc7ce22c7f87c253$$aChang-Claude, Jenny$$b22 000306176 7001_ $$aNBCS Collaborators$$b23$$eCollaboration Author 000306176 7001_ $$aCzene, Kamila$$b24 000306176 7001_ $$aDennis, Joe$$b25 000306176 7001_ $$aDevilee, Peter$$b26 000306176 7001_ $$aDörk, Thilo$$b27 000306176 7001_ $$aDunning, Alison M$$b28 000306176 7001_ $$aEriksson, Mikael$$b29 000306176 7001_ $$aEvans, D Gareth$$b30 000306176 7001_ $$aFasching, Peter A$$b31 000306176 7001_ $$aFigueroa, Jonine D$$b32 000306176 7001_ $$aGabrielson, Marike$$b33 000306176 7001_ $$aGago-Dominguez, Manuela$$b34 000306176 7001_ $$aGonzález-Neira, Anna$$b35 000306176 7001_ $$aGuénel, Pascal$$b36 000306176 7001_ $$aHadjisavvas, Andreas$$b37 000306176 7001_ $$aHahnen, Eric$$b38 000306176 7001_ $$0P:(DE-He78)537e07b3e57b16c7b214fc2242e4326b$$aHamann, Ute$$b39 000306176 7001_ $$aHillemanns, Peter$$b40 000306176 7001_ $$aHollestelle, Antoinette$$b41 000306176 7001_ $$aHooning, Maartje J$$b42 000306176 7001_ $$aHoppe, Reiner$$b43 000306176 7001_ $$aHowell, Anthony$$b44 000306176 7001_ $$aInvestigators, kConFab$$b45$$eCollaboration Author 000306176 7001_ $$aJakubowska, Anna$$b46 000306176 7001_ $$aKristensen, Vessela N$$b47 000306176 7001_ $$aLubiński, Jan$$b48 000306176 7001_ $$aLush, Michael$$b49 000306176 7001_ $$aManoukian, Siranoush$$b50 000306176 7001_ $$aMavroudis, Dimitrios$$b51 000306176 7001_ $$aMilne, Roger L$$b52 000306176 7001_ $$aMulligan, Anna Marie$$b53 000306176 7001_ $$aNewman, William G$$b54 000306176 7001_ $$aObi, Nadia$$b55 000306176 7001_ $$aPanayiotidis, Mihalis I$$b56 000306176 7001_ $$aPita, Guillermo$$b57 000306176 7001_ $$0P:(DE-HGF)0$$aRashid, Muhammad U$$b58 000306176 7001_ $$aRhenius, Valerie$$b59 000306176 7001_ $$aSaloustros, Emmanouil$$b60 000306176 7001_ $$aSawyer, Elinor J$$b61 000306176 7001_ $$aSchmutzler, Rita K$$b62 000306176 7001_ $$aShah, Mitul$$b63 000306176 7001_ $$aSouthey, Melissa C$$b64 000306176 7001_ $$aSpurdle, Amanda B$$b65 000306176 7001_ $$aTomlinson, Ian$$b66 000306176 7001_ $$aTruong, Thérèse$$b67 000306176 7001_ $$aWang, Qin$$b68 000306176 7001_ $$aWendt, Camilla$$b69 000306176 7001_ $$aAuer, Paul L$$b70 000306176 7001_ $$aBoddicker, Nicholas J$$b71 000306176 7001_ $$aBodelon, Clara$$b72 000306176 7001_ $$aBurnside, Elizabeth S$$b73 000306176 7001_ $$aChen, Fei$$b74 000306176 7001_ $$aCouch, Fergus J$$b75 000306176 7001_ $$aDomchek, Susan M$$b76 000306176 7001_ $$aEliassen, Heather A$$b77 000306176 7001_ $$aHaiman, 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