Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000987427 | SCV001136717 | pathogenic | Retinitis pigmentosa 41 | 2019-05-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001865639 | SCV002243660 | pathogenic | not provided | 2022-05-28 | criteria provided, single submitter | clinical testing | This variant is present in population databases (rs780697796, gnomAD 0.01%). For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. ClinVar contains an entry for this variant (Variation ID: 438214). This premature translational stop signal has been observed in individuals with autosomal recessive PROM1-related conditions (PMID: 25356976, 28041643, 29555955). This sequence change creates a premature translational stop signal (p.Arg146*) 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). |
NIHR Bioresource Rare Diseases, |
RCV000504986 | SCV000599177 | pathogenic | Retinal dystrophy | 2015-01-01 | no assertion criteria provided | research |