Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genetic Services Laboratory, |
RCV001822609 | SCV002067251 | uncertain significance | not specified | 2021-07-29 | criteria provided, single submitter | clinical testing | DNA sequence analysis of the ATM gene demonstrated a sequence change, c.5546T>C, in exon 37 that results in an amino acid change, p.Ile1849Thr. This sequence change has not been described in population databases including gnomAD. The p.Ile1849Thr change affects a highly conserved amino acid residue located in a domain of the ATM protein that is known to be functional. The p.Ile1849Thr substitution appears to be deleterious using several in-silico pathogenicity prediction tools (SIFT, PolyPhen2, Align GVGD, REVEL). This sequence change does not appear to have been previously described in individuals with ATM-related disorders. Due to insufficient evidences and the lack of functional studies, the clinical significance of the p.Ile1849Thr change remains unknown at this time. |