Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001008873 | SCV001168679 | pathogenic | not provided | 2019-02-20 | criteria provided, single submitter | clinical testing | This duplication of four nucleotides in BRCA2 is denoted c.1794_1797dupATCT at the cDNA level and p.Tyr600IlefsX3 (Y600IfsX3) at the protein level. Using alternate nomenclature, this variant would also be defined as BRCA2 2022_2025dupATCT. The normal sequence, with the bases that are duplicated in brackets, is AAAC[dupATCT]TATA. The duplication causes a frameshift which changes a Tyrosine to an Isoleucine at codon 600, and creates a premature stop codon at position 3 of the new reading frame. Although this variant has not, to our knowledge, been reported in the literature as a germline variant, it is predicted to cause loss of normal protein function through either protein truncation or nonsense-mediated mRNA decay. We consider this variant to be pathogenic. |