Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV003317737 | SCV004020589 | pathogenic | Tuberous sclerosis syndrome | 2023-06-26 | criteria provided, single submitter | clinical testing | Variant summary: TSC1 c.792_793dupGG (p.Asp265GlyfsX54) results in a premature termination codon, predicted to cause absence of the protein due to nonsense mediated decay, which is a commonly known mechanism for disease. The variant was absent in 251438 control chromosomes. To our knowledge, no occurrence of c.792_793dupGG in individuals affected with TSC1-related conditions and no experimental evidence demonstrating its impact on protein function have been reported. No submitters have cited clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as pathogenic. |