Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001058613 | SCV001223197 | pathogenic | not provided | 2022-10-24 | criteria provided, single submitter | clinical testing | This premature translational stop signal has been observed in individual(s) with Usher syndrome type II (PMID: 25558175, 25575603). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. This variant is present in population databases (rs767328784, gnomAD 0.0009%). ClinVar contains an entry for this variant (Variation ID: 853741). This sequence change creates a premature translational stop signal (p.Met3271Cysfs*30) in the USH2A gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in USH2A are known to be pathogenic (PMID: 10729113, 10909849, 20507924, 25649381). For these reasons, this variant has been classified as Pathogenic. |
Baylor Genetics | RCV003467794 | SCV004206285 | pathogenic | Retinitis pigmentosa 39 | 2023-03-16 | criteria provided, single submitter | clinical testing |