Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Medical Genetics and Applied Genomics, |
RCV001268612 | SCV001447657 | pathogenic | not provided | 2020-10-23 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001880169 | SCV002194698 | pathogenic | Dyskeratosis congenita | 2023-01-30 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This sequence change creates a premature translational stop signal (p.Arg224*) in the CTC1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CTC1 are known to be pathogenic (PMID: 22267198, 22387016). This variant is present in population databases (no rsID available, gnomAD 0.002%). This variant has not been reported in the literature in individuals affected with CTC1-related conditions. ClinVar contains an entry for this variant (Variation ID: 987292). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. |
Prevention |
RCV003898254 | SCV004710246 | pathogenic | CTC1-related disorder | 2023-11-10 | no assertion criteria provided | clinical testing | The CTC1 c.670C>T variant is predicted to result in premature protein termination (p.Arg224*). To our knowledge, this variant has not been reported in the literature in a patient with a CTC1-related disorder. This variant is reported in 0.0018% of alleles in individuals of European (Non-Finnish) descent in gnomAD (http://gnomad.broadinstitute.org/variant/17-8140815-G-A). Nonsense variants in CTC1 are expected to be pathogenic. This variant is interpreted as pathogenic. |