Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000591097 | SCV000706394 | pathogenic | not provided | 2017-04-11 | criteria provided, single submitter | clinical testing | |
Blueprint Genetics | RCV001074705 | SCV001240298 | pathogenic | Retinal dystrophy | 2019-04-03 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000591097 | SCV001590236 | pathogenic | not provided | 2023-06-11 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 500442). This variant has not been reported in the literature in individuals affected with PROM1-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Tyr412Metfs*34) in the PROM1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in PROM1 are known to be pathogenic (PMID: 17605048, 19718270, 24154662, 25474345). |
Prevention |
RCV004530703 | SCV004117953 | likely pathogenic | PROM1-related disorder | 2023-07-05 | criteria provided, single submitter | clinical testing | The PROM1 c.1234delT variant is predicted to result in a frameshift and premature protein termination (p.Tyr412Metfs*34). To our knowledge, this variant has not been reported in the literature or in a large population database (http://gnomad.broadinstitute.org), indicating this variant is rare. Frameshift variants in PROM1 are expected to be pathogenic. Therefore we interpret c.1234del (p.Tyr412Metfs*34) as likely pathogenic. |