Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV004582704 | SCV005062294 | likely pathogenic | Tuberous sclerosis 2 | 2018-08-07 | criteria provided, single submitter | clinical testing | This variant is a gross deletion of the genomic region encompassing exon 11 and part of exon 12 (c.976-207_1191del) of the TSC2 gene. It is expected to disrupt RNA splicing and likely results in an absent or disrupted protein product. This variant has not been reported in the literature in individuals with TSC2-related disease. In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. Loss-of-function variants in TSC2 are known to be pathogenic (PMID: 10205261, 17304050). |