Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001033658 | SCV001196965 | pathogenic | Drash syndrome; Frasier syndrome; Wilms tumor 1; 11p partial monosomy syndrome | 2019-04-20 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in WT1 are known to be pathogenic (PMID: 15150775). A similar variant has been observed de novo in an individual affected with early onset unilateral nephroblastoma (PMID: 1350671). This variant is an out-of-frame deletion of the genomic region encompassing exons 6 and 7 of the WT1 gene. This is expected to create a premature translational stop signal and result in an absent or disrupted protein product. |