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. 2022 Apr 2;21(4):489–555. doi: 10.1007/s10689-021-00284-8

Table 1.

ATM mutations detected in colorectal tumors from patients with known pathogenic germline ATM mutations

Patient Germline ATM Mutation VAF in Tumor Somatic ATM Mutation VAF in Tumor Tumor Content Tumor Supports ATM In Tumorigenesis?
1 p.E2052K, c.6154G > A^ 50% p.R2227C, c.6679C > T 26% 45% Yes
2 p.F2799Kfs*4,c.8395_8404del 24%* c.4611 + 4A > G 23% 50% Yes
3** p.R2993*, c.8977C > T 49% p.Y741C, c.2222A > G 21% 40% Yes
4 p.Q522Ifs*43, c.1564_1565del 78% Loss of Heterozygosity NA 60% Yes
5 p.S1905Ifs*25, c.5712dup 79% Loss of Heterozygosity NA 70% Yes
6*** p.V2424G, c.7271 T > G 82% Loss of Heterozygosity NA 80% Yes

^Germline classification is conflicting depending on laboratory (ranging to variant of uncertain significance to Pathogenic)

*VAF was identical in germline and tumor samples at 24%. Reference allele bias accounts for the lower than expected VAF

**Patient 3 also carried a germline pathogenic NBN Slavic Founder mutation, p.K219Nfs*16, c.657_661del

***Patient 6 also carried a germline pathogenic CHEK2 p.T367Mfs*15, c.1100delC mutation

VAF = variant allele fraction

NM_000051.3 is the ATM reference transcript used for mutation annotations