Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genetic Services Laboratory, |
RCV000500806 | SCV000594260 | pathogenic | Coats plus syndrome | 2016-05-13 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002524173 | SCV003477971 | pathogenic | Dyskeratosis congenita | 2022-09-28 | criteria provided, single submitter | clinical testing | This premature translational stop signal has been observed in individual(s) with cerebroretinal microangiopathy with calcifications and cysts and/or paroxysmal nocturnal haemoglobinuria (PMID: 23220793, 30891747). For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 434858). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Asp405Metfs*58) 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). |